Peds Flashcards

Question

LCH

Answer 1

(Langerhans Cell Histiocytosis) - Too many fucking dendritic cells - with local invasion. It is a sorta pseudo malignancy thing. Nobody really understands it.... For the purpose of the exam think about this as a beveled hole in the skull. The skull is the most common bone involved with LCH. It is a pure lytic lesion (no sclerotic border). The beveled look is because it favors the inner table. It can also produce a sequestrum o f intact bone (“button sequestrum).

Answer 2

If they tell you (or infer) the kid has neuroblastoma | - think about a met.

Answer 3

These paired, mostly round, defects in the parietal bones represent benign congenital defects. The underlying cause is a delayed or incomplete ossification in the underlying parietal bones. They can get big and confluent across the midline. Supposedly, (at least for the big ones - > 5mm) they are associations with cortical and venous anomalies.

Answer 4

In technical terms, there are a bunch o f extra squiggles around the lambdoid sutures. “Intrasutural Bones” they call them. These things are usually idiopathic - however, if you see more than 10 you should start thinking syndromes.

Answer 5

< 10 = Idiopathic > 10 = First think Osteogenesis Imperfecta > 10 + Absent Clavicle = Cleidocranial Dysostosis

Answer 6

There is a massive differential, but 1 would just remember these “PORK-CHOP” ``` Pyknodysostosis Osteogenesis Imperfecta Rickets Kinky Hair Syndrome (Menke s / Fucked Copper Metabolism) Cleidocranial Dysostosis Hypothyroidism / Hypophosphatasia One too many 21 st chromosomes (Downs) Primary Acro-osteolysis (Hajdu-Cheney) ```

Answer 7

``` In the context of the skull, you can think about these things as occurring from the congenital misplacement of cells from the scalp into the bony calvarium. The result is a growing lump o f tissue (keratin debris, skin glands, etc...) creating a bone defect with benign appearing sclerotic borders. There are a few differences between the two subtypes that could be potentially testable (contrasted masterfully in the chart). ``` ``` Although, I suspect a “what is it ? ” type question is more likely. As such, look through some google image examples to prepare yourself for that contingency. ```

Answer 8

Histology:Only Skin (Squamous Epithelium) Age of onset:Present between age 20-40 Location: Present between age 20-40 CT: CSF Density MRI:T1 Variable, T2 Bright, NO Enhancement

Answer 9

Histology: Skin + Other Stuff Like Hair Follicles, Sweat Glands Etc Age of onset:Typically have an earlier presentation Location: end to be midline. The skin ones tend to he around the orbits. Associated with Encephaloceles - especially when midline CT: - More Heterogeneous, - Calcifications (internal or peripheral) may be present MRI: T2 Bright, +/- Wall Enhancement

Answer 10

Usually when people talk about these things they are referring to the spina bifida style midline lumbosacral region defects. However, we are going to stay focused on the skull / face. The two classic locations for dermal communications with the dura are the occiput and the nose. Both of which are classically midline, and can be associated with a dermoid cyst. For gamesmanship, consider a sinus tract anytime you see a cyst in these locations. Dural communication will require communication through the foramen cecum. ``` Sinus tracts may or may not have associated dennal or intraosseous cysts. Cysts may or may not have sinus tracts ```

Answer 11

There are 3 scalp hematoma subtypes. Because the subtypes are fairly similar, there is a high likelihood a sadistic multiple choice writer will attempt to confuse you on the subtle differences

Answer 12

Location:Deep to the Aponeurosis (between aponeurosis and periosteum) Suture:NOT limited by suture lines Trivia: Covers a much larger area than a cephalohematoma Complications:Potentially life-threatening - rapid blood loss. Often not seen until 12-72 hours post delivery. Cause: vacuum extraction

Answer 13

Location: Under the Periosteum (skin o f the bone) Suture: Limited by suture lines (won i cross sutures) Trivia:Outer border may calcify as a rim and leave a deformity - sorta like a myositis ossificans ``` Complications:Usually requires no intervention (resolves within a few weeks) Can get super infected (E.Coli). Abscess would require drainage. Can cause skull osteomyelitis ``` Cause:Instrument or Vacuum Extraction

Answer 14

Location:Subcutaneous Hemorrhage (superficial to the aponeurosis) Suture:NOT limited by suture lines Trivia:Requires no intervention (resolves within a few days) Cause: prolonged delivery

Answer 15

Accidental (and non-accidental) head trauma is supposedly (allegedly, allegedly) the most common cause of morbidity and mortality in children. As you might imagine, the pediatric skull can fracture just like the adult skull - with linear and comminuted patterns. For the purpose of multiple choice, I think we should focus on the fracture patterns that are more unique to the pediatric population: Diastatic, Depressed, and “Ping-Pong”

Answer 16

This is a fracture along / involving the suture. When they intersect it is usually fairly obvious. It can get tricky when the fracture is confined to the suture itself. The most common victim of this sneaky fracture is usually the Lambdoid, followed by the Resident reading the case on night float .. .with Attending backup (asleep in bed). How does one know there is traumatic injury to a suture ? Classically, it will widen. This is most likely to be shown in the axial or coronal plane so you can appreciate the asymmetry ( > 1 mm asymmetry relative to the other side).

Answer 17

This is a fracture with inward displacement of the bone. How much inward displacement do you need to call it “depressed” ? Most people will say “equal or greater to the thickness of the skull.” Some people will use the word “compound” to describe a depressed fracture that also has an associated scalp laceration. Those same people may (or may not) add the word "penetrating” to describe a compound fracture with an associated dural tear. Will any o f those people be writing the questions ? The dark side clouds everything. Impossible to see the future is.

Answer 18

Ping Pong fractures typically have a favorable / benign clinical outcome (depressed fractures have high morbidity).

Answer 19

Diastatic and depressed fracture types usually require a significant wack on the head. Where as “ping pong” fractures often occur in the setting of birth trauma (Mom’s pelvic bones +/- forceps).

Answer 20

Ping Pong fractures are hard as fuck to see. To show this on a test you’d have to have CT 3D recons demonstrating a smooth inward deformity. You could never see that shit on a plain film. I can’t imagine anyone being a big enough asshole to ask you to do that. Hmmm.... probably.

Answer 21

This is actually another subtype of depressed fracture but is unique in that it is a greenstick or “buckle” type of fracture.

Answer 22

Unlike linear fractures (which usually heal without complication), depressed fractures often require surgery. Some general indications Fracture for surgery would include: Depression of the fragments > 5mm (supposedly fragments more than 5mm below the inner table are associated with dural tears), Epidural bleed Superinfection (abscess, osteomyelitis) “Form” (cosmetic correction to avoid looking like a gargoyle), “Function” (if the frontal sinus is involved, sometimes they need to obliterate the thing to avoid mucocele formation).

Answer 23

``` Although car wrecks and falls account for the majority o f skull fractures in children, there still remains the timeless truth — some people just can’t take screaming kids. For the purpose of multiple choice, the follow clues should make your spider-sense tingle. • Inconsistent History: “My 7 month old wrecked his bike ” • Subdural Hematoma • Retinal hemorrhage • DAI / Parenchymal Contusion • Cerebral Edema, Stroke (less specific but still worrisome) • Depressed Skull Fx, or Fracture Crossing Suture Line (less specific but still worrisome) ```

Answer 24

Subdurals have a stronger association with NAT relative to epidurals. Think about vigorous shaking (trying to get that last drop of ketchup out of the bottle) tearing bridging cortical veins. “Look High, Look Low” - Sneaky Ways to Suggest NAT - - Look High: Thrombosed (hyperdense) cortical vein at the vertex - Look Low: Retroclival hematoma (thin hyperdense sliver in the pre-pontine region) - Look Lower: Edema within the cervical soft tissues

Answer 25

Epidural Below the Tectorial Membrane Subdural Above the tectorial Membrane

Answer 26

Chronic Subdural (CSF Density) Medial Displacement of Bridging Vein (sometimes smashed and not well seen). Usually Unilateral. If Bilateral Usually Asymmetric in Size. Prominent CSF Spaces Cortical veins are adjacent to the inner table Usually Symmetric

Answer 27

Extra-axial fluid spaces are considered enlarged if they are greater than 5 mm. BESSI is the name people throw around for “benign enlargement of the subarachnoid space in infancy.’ The etiology is supposed to be immature villa (that’s why you grow out of it).

Answer 28

``` BESSI - Cortical veins are adjacent to the inner table - they are usually seen secondary to enlargement o f the subarachnoid spaces (positive cortical vein sign) Subdural - Cortical veins are displaced away from the inner table - they are often not se ```

Answer 29

• It’s the most common cause of macrocephaly, • Typically presents around month 2 or 3, and has a strong male predominance. • Typically resolves after 2 years with no treatment, • There is an increased risk of subdural bleed - either spontaneous or with a minor trauma. This subdural is usually isolated (all the same blood age), which helps differentiate it from nonaccidental trauma, where the bleeds are often of different ages.

Answer 30

``` Pre-mature kids getting tortured on ECMO often get enlarged extra-axial spaces. This isn’t really the same thing as BESSI but rather more related to fluid changes / stress. ```

Answer 31

Enlarged symmetric subarachnoid spaces favoring the anterior aspect o f the brain (spaces along the posterior aspect o f the brain are typically normal). Brain parenchyma is normal and there is either normal ventricle size or very mild communicating hydrocephalus. Communicating meaning that all 4 ventricles are big.

Answer 32

This is the result of an ischemic / hemorrhagic injury, typically from a hypoxic insult during birthing. The kids who are at the greatest risk are premature and little (less than 1500 g). The testable stigmata is cerebral palsy - which supposedly develops in 50%. The pathology favors the watershed areas (characteristically the white matter dorsal and lateral to the lateral ventricles).

Answer 33

The milder finding can be very subtle. Here are some tricks: (1) Use PreTest Probability: The kid is described as premature or low birth weight. (2) Brighter than the Choroid: The choroid plexus is an excellent internal control. The normal white matter should always be less bright (less hyperechoic) when compared to the choroid (3) "Blush” and "Flaring” : These are two potential distractors that need to be differentiated from legit grade 1 PVL. "Blush ” describes the physiologic brightness of the posterosuperior periventricular white matter - this should be less bright than choroid, and have a more symmetric look. “Flaring” is similar to blush, but a more hedgy term. It’s the word you use if you aren’t sure if it’s real PVL or just the normal brightness often seen in premature infants white matter. The distinction is that "flaring” should go away in a week. Grade 1 PVL persists > 7 days.

Answer 34

Early: P e riv en tric u la r W h ite M a tte r N e c ro sis (hyperechoic relative to choroid) S u b acu te: C y s t F o rm a tio n

Answer 35

The most severe grade (4), which has subcortical cysts, is actually more common in full term infants rather than preterms.

Answer 36

The later findings are more obvious with the development of cavitary periventricular cysts. The degree of severity is described by the size and distribution of these cysts. These things take a while to develop - some people say up to 4 weeks. So, if they show you a day 1 newborn with cystic PVL they are leading you to conclude that the vascular insult occurred at least 2 weeks prior to birth (not during birth - which is often the case).

Answer 37

I like to think about the germinal matrix as an embryologic seed that sprouts out various development cells during brain development. Just like a seed needs water to grow, the germinal matrix is highly vascular. It’s also very friably and susceptible to stress. Additionally, premature brains suck at cerebral blood flow auto-regulation. Mechanism: Fragile vessels + too much pressure/flow = bleeds An important thing to understand is that the germinal matrix is an embryological entity. So it only exists in premature infants. As the fetus matures the thing regresses and disappears. By 32 weeks, germinal matrix is only present at the caudothalamic groove. By 36 weeks, you basically can’t have it (if no GM, then no GM hemorrhage).

Answer 38

No GM Hemorrhage in a full term infant.

Answer 39

Similar looking bleed in a full term infant say “choroid plexus hemorrhage” (not GMH).

Answer 40

The scenario will always call the kid a premature infant (probably earlier than 30 weeks). The earlier they are bom the more common it is. Up to 40% occur in the first 5 hours, and most have occurred by day 4 (90%). A good thing to remember is that 90% occur in the first week.

Answer 41

Head US is used to screen for this pathology. Testable trivia includes: • Who should be screened? Premature Infants (<32 weeks, < 1500 grams), Premature Infants with Lethargy, Seizures, Decreased Hematocrit or a history o f “he don’t look so good.” • When do you do the head US? First week o f life (remember this is when 90% o f them occur). Some people will tell you - “first week and first month” (but that varies from institution). Some people will also say - “every kid gets a head US prior to discharge from the NICU” - but that is mainly done to detect PVL (not necessarily GMH).

Answer 42

Choroid Plexus is bright (hyperechoic) on ultrasound. Blood is also bright (hyperechoic). You tell them apart based on their location. Choroid should not extend anterior to the junction o f the caudate and the thalamus (the so called caudothalamic groove). This is the location o f the germinal matrix. If you see bright stuff there - that is your grade 1 bleed. This could also be shown with MR1 (T1 bright = bleed), same location.

Answer 43

``` 1 - Blood at the caudothalamic groove 2 - Blood in the ventricles but no dilation. 3 - Blood in the ventricles with dilation 4 - Blood in the brain parenchyma (from venous infarct) ```

Answer 44

* Caudothalamic groove in the coronal plane * Grade 2,3,4 Bleeds * Subependymal Cysts on US * Porencephalic Cysts on US * Choroid Plexus Cysts on US * Ventricular coarctation on US - this is a mimic

Answer 45

Subependymal hemorrhage either results in a subependymal cyst or resolution or to a grade 2

Answer 46

Intraventricular hemorrhage results in hydrocephalus or grade 4

Answer 47

intraparenchymal hemorrhage either goes to a porencephalic cyst or hydrocephalus.

Answer 48

Results from a membrane that separates the nasal cavity from its normal communication with the oral cavity. It is usually unilateral but can be bilateral. The bilateral ones have a history of “cyclical cyanosis that improves with crying ” (they mouth breath when they cry). The unilateral classic stories are “can't pass NG tube, ” and “respiratory distress while feeding” (neonates have to breath through their noses). You will also sometimes hear a history of “a continuous stream o f snot draining from one or both nostrils, ” or the word “rhinorrhea. ”

Answer 49

There are two different types: bony (90%), and membranous (10%). The appearance is a unilateral or bilateral posterior nasal narrowing, with thickening of the vomer.

Answer 50

There are many syndromic associations including CHARGE. Crouzons, DiGeorge, Treacher Collins, and Fetal Alcohol Syndrome. CHARGE is the one people mention the most.

Answer 51

``` Coloboma, Heart defect, Atresia (Choanal) Retarded growth, Genitourinary abnormalities Ear anomalies ```

Answer 52

This results from abnormal development of the medial nasal eminences, and subsequent failure of formation of the primary palate. You can see this in isolation or with choanal atresia. The piriform aperture of the nasal cavity (bony inlet of the nose) is stenotic (as the name suggests), and the palate is narrow. The classic picture is the associated central maxillary “MEGA-incisor.” Midlinc defects of the brain (corpus callosum agenesis, and holoprosencephaly) are associated — as my Grandma always said “face predicts brain ”.

Answer 53

You have to image the brain

Answer 54

The big thing to know is the high association with hypothalamicpituitary- adrenal axis dysfunction.

Answer 55

Thyroid topics will be covered again in the endocrine chapter. 1 do want to mention one or two now for completeness. To understand ectopic thyroid trivia you need to remember that the thyroid starts (embryology wise) at the back of the tongue. It then descends downward to a location that would be considered normal. The “pyramidal lobe” actually represents a persistence of the inferior portion of the thyroglossal duct - that is why this thing is so variable in appearance. Sometimes this process gets all fucked up and the thyroid either stays at the back of the tongue (lingual thyroid) or ends up half way down the neck or even in the chest (ectopic thyroid).

Answer 56

* Most “developed” countries test for low thyroid at birth (Guthrie Test). That will trigger a workup for either ectopic tissue or enzyme deficiencies. * Nukes (1-123 or Tc-MIBI) is superior to ultrasound for diagnosing ectopic tissue. This is by far the most likely way to show this on a multiple choice exam. I guess CT would be #2 - remember thyroid tissue is dense because of the iodine. * Ultrasound does have a preoperative role in any MIDLINE neck mass - with the point of ultrasound being to confirm that you have a normal thyroid in a normal place. If you resect a midline mass (which turns out to be the kids only thyroid tissue) you can expect an expensive well rehearsed didactic lecture on pediatric neck pathology from an “Expert Witness” sporting a $500 haircut. * Lingual thyroid (back of the tongue) is the most common location of ectopic thyroid tissue

Answer 57

As we discussed previously, thyroid related pathology can occur anywhere between the foramen cecum (the base of the tongue) and the thyroid gland. In this situation we are talking about the duct (which is the embryological thyroid interstate highway to the neck) failing to involute fully. What you get is a left over cyst - hence the name. The classic locations are (1) at the base of the tongue, and (2) midline anterior to the hyoid. Now textbooks will make a big deal about these things becoming slightly lateral below the hyoid. Do NOT get hung up on that. For the purpose of multiple choice remember these guys are midline. Midline is the buzzword.

Answer 58

* Classic Buzzword / Scenario = Midline Cyst in the Neck o f a Kid. * Next step once you find one = confirm normal thyroid location and/or look for ectopic tissue (Ultrasound +/-Tc-M1BI, or 1-123). * They are cystic (it’s not called a “Duct Solid”) * Enhancing nodule within the cyst = CANCER (usually papillary) * They can get infected.

Answer 59

It is true that dermoids almost always occur below the clavicles, but when they do happen in the neck they have a pretty classic look: midline sublingual / submandibular space with a “sac of marbles” appearance. The marbles are lobules of fat within fluid.

Answer 60

Another cystic embryologic remnant. There are a bunch o f types (and subtypes... and subsubtypes) and you can lose your fucking mind trying to remember all o f them - don’t do that. Just remember that by far the most common is a 2nd Branchial Cleft Cyst (95%). The angle of the mandible is a classic location. They can get infected, but are often asymptomatic. Extension o f the cyst between the ICA and ECA (notch sign) just above the carotid bifurcation is pathognomonic.

Answer 61

Most likely on CT or MR1. Ultrasound would be tough, unless they clearly labeled the area “lateral neck” or oriented you in some other way.

Answer 62

``` They could (and this would be super mean) ask you the relationship o f a type 2 based on other neck anatomy. So - posterior and lateral to the submandibular gland or lateral to the carotid space, or anterior to the sternocleidomastoid. How I would handle that? Just remember it’s going to be lateral to everything. Lateral is the buzzword ```

Answer 63

``` They could try and trick you into calling a necrotic level 2 lymph node a BCC. Thyroid cancer (history o f radiation exposure) and nasopharyngeal cancer (history ofHPV) can occur in “early adulthood.” If you have a “new” BCC in an 18 year old - it’s probably a necrotic node. Next Step = Find the cancer +/- biopsy the mother fucker. ```

Answer 64

branchial cleft cyst

Answer 65

thyroglossal duct cyst

Answer 66

clotted jugular vein. You see this classically in the setting of a recent pharyngeal infection (or recent ENT surgery).

Answer 67

Showing the clotted vein with the appropriate clinical history.

Answer 68

Seeing if you know that it has a fancy syndrome name.

Answer 69

Looking in the lungs for septic emboli. This could also be done in the reverse. Show you the septic emboli, give you a history of ENT procedure (or recent infection), and have you ask for the US of the neck veins.

Answer 70

Fusobacterium necrophorum is the bacteria that causes the septic emboli. As this bacteria sounds like a Marvel Comic villain the likelihood of it being asked increases by at least 5x.

Answer 71

Idiopathic dilated jugular vein.

Answer 72

showing the dilated vein

Answer 73

is it not related to a stenosis. there aren o other signs of venous congestion

Answer 74

It gets worse with the Valsalva maneuver - “neck mass that enlarges with valsalva. ”

Answer 75

If you must try and tell them apart - you could try this: • Venous Malformations will have enhancement of the cystic spaces. • Lymphatic Malformations will have enhancement of the septa. • Phlcboliths — suggests venous.

Answer 76

These things are actually the most common congenital lesions in the head and neck. Just like any hemangioma they contain vascular spaces with varying sizes and shapes. Most people consider them a “tumor” more than a vascular malformation.

Answer 77

Super T2 bright, with a bunch of | flow voids. Diffusely vascular on doppler.

Answer 78

Typically they show up around 6 months of age, grow for a bit, then plateau, then involute (6-10 years). Usually they require no treatment.

Answer 79

Large size / Rapid growth | with mass effect on the airway or adjacent vascular structures. Fucking with the kids eye movement or eyelid opening.

Answer 80

Typically medical = Beta blocker | propranolol

Answer 81

``` PHACES Syndrome (discussed later in the chapter) ```

Answer 82

This is another cystic lesion o f the neck, which is most likely to be shown as an OB ultrasound (but can occur in the Peds setting as well). The classic look / location is a cystic mass hanging off the back o f the neck on OB US (or in the posterior triangle if CT/MR1).

Answer 83

Turners (most common association). Downs (second most common association). ``` Aortic Coarctation (most common CV abnormality), Fetal Hydrops (bad bad bad outcomes). ```

Answer 84

worse outcome

Answer 85

``` T2 Bright (like a hemangioma). Does NOT enhance (hemangiomas typically do). ```

Answer 86

This is a benign “mass” of the sternocleidomastoid in neonates who present with torticollis (chin points towards the opposite side - or you could say they look away from the lesion). It’s really just a benign inflammation that makes the muscle look crazy big. Ultrasound can look scary, until you realize it’s just the enlarged SCM. Ultrasound is still the best imaging test. Sometimes it looks like there are two of them, but that’s because the SCM has two heads. It goes away on its own, sometimes they do passive physical therapy or try and botox them.

Answer 87

• Most common cause of a neck “mass” in infancy • Classic scenario is a 4 week old with a palpable neck mass and torticollis toward the affected side • Best imaging test = US. • Things that make you think it’s not FC: mass is outside the SCM, or internal calcifications - in which case you should think to yourself... nice try Mother fuckers- that’s a neuroblastoma.

Answer 88

So... there can be significant fuckery with the “direction” things curve or people look depending on how the question is ask. What do I mean ? If you made the mistake of just memorizing the word “towards” in association with fibromatosis coli you might get tricked if the options were: A - Patient looks towards the involved side. B - Patient looks toward the uninvolved side. You’d run into the same problem with the word “away.” Now, that might seem obvious once I spell it out like that but I’m pretty sure at least a few of you were making a flashcard that had only the word “towards” on it. You have to assume the test writer has the worst intentions for you. Don’t provide them with any opportunity to trick you.

Answer 89

Although technically rare as fuck, this is the most common mass in the masticator space of a kid. Having said that if you see it in the head/neck region is almost always in the orbit. In fact, its the most common extra-occular orbital malignancy in children (dermoid is most common benign orbital mass in child). The most classic scenario would be an 8 year old with painless proptosis and no signs of infection.

Answer 90

I'll talk about this more in the MSK chapter, but in general I’ll just say they look mean as cat shit (enhancing, solid, areas of necrosis, etc..).

Answer 91

This is the most common cause o f acute upper airway obstruction in young children. The peak incidence is between 6 months and 3 years (average 1 year). They have a barky “croupy” cough. It’s viral. The thing to realize is that the lateral and frontal neck x-ray is done not to diagnosis croup, but to exclude something else. Having said that, the so-called “steeple sign ” - with loss o f the normal lateral convexities o f the subglottic trachea is your buzzword, and if it’s shown, that will be the finding. Questions are still more likely to center around facts (age and etiology).

Answer 92

The culprit is often parainfluenza virus.

Answer 93

In contrast to the self-limited croup, this one can kill you. It’s mediated by H. Influenza and the classic age is 3.5 years old (there is a recent increase in teenagers - so don’t be fooled by that age). The lateral x-ray will show marked swelling o f the epiglottis (thumb sign). A fake out is the “omega epiglottis” which is caused by oblique imaging. You can look for thickening o f the aryepiglottic folds to distinguish.

Answer 94

Death by asphyxiation is from the aryepiglotic folds (not the epiglottis)

Answer 95

This is an uncommon but serious (possibly deadly) situation that is found in slightly older kids. It’s caused by an exudative infection o f the trachea (sorta like diptheria). It’s usually from Staph A. and affects kids between 6-10.

Answer 96

linear soft tissue filling defect | within the airway.

Answer 97

6 months - 3 years (peak 1 year) Steeple Sign: loss o f the normal shoulders (lateral convexities) of the subglottic trachea Viral (Most Common - parainfluenza)

Answer 98

Classic = 3.5 years, but now seen with teenagers too Thumb Sign: marked enlargement o f epiglottis h. flu

Answer 99

6-10 years Linear soft tissue filling defect (a membrane) seen within the airway Staph A

Answer 100

I’ll just say quickly that you do see this most commonly in young kids (age 6 months -12 months). If they don't show it on CT, they could show it with a lateral x-ray demonstrating massive retropharyngeal soft tissue thickening. For the real world, you can get pseudothickcning when the neck is not truly lateral To tell the difference between positioning and the real thing, a repeat with an extended neck is the next step.

Answer 101

Hemangiomas are the most common soft tissue mass in the trachea, and they are most commonly located in the subglott region. In croup there is symmetric narrowing with loss of shoulders on both sides (Steeple Sign). In contradistinction, subglottic hemangiomas have loss of just one o f the sides.

Answer 102

* Tends to favor the left side * 50% are associated with cutaneous hemangiomas * 7% have the PHACES syndrome

Answer 103

``` P- Posterior fossa (Dandy Walker) H- Hemangiomas A- Arterial anomalies C- Coarctation o f aorta, cardiac defects E- Eye abnormalities S- Subglottic hemangiomas ```

Answer 104

This is a zebra. The classic scenario is contrast appearing in the tracheal without laryngeal penetration (aspiration). They could also show you a “thin tract of contrast extending to the larynx or trachea.” This entity is a communicating defect in the posterior wall of the larynx and the esophagus or anterior hypo pharynx. There arc a bunch of different cleft classifications - 1 can’t imagine that shit is appropriate for the exam.

Answer 105

• This is a thing - maybe google a Fluoro swallow picture of it • These things have other complex malformations associated with them (usually GI) • It is very tricky to call it with certainty on a swallow exam - definite diagnosis is always made with direct visualization / scope (so a next step type question would recommend endoscopy to confirm).

Answer 106

If you see a lobulated grape looking thing in the airway - think Papilloma, especially if the lungs are full of nodules (solid and cavitated). When 1 say Papilloma, You say HPV - typically from perinatal (birth canal) transmission. These things are usually multiple (papillomatosis) and therefore have multiple areas of airspace disease (atelectasis ect.). Some potential gamesmanship — because these thing are typically multiple you will have more areas of air trapping then you would compared with an aspirated crayon (or green bean), or even a solitary endobronchial lesion like a carcinoid. Multiple areas of air trapping - think Papillomatosis over carcinoid or a foreign body. Having said that the nodules are a more common finding... lots of them.

Answer 107

(1) Croup and (2) Subglottic Hemangioma You can tell them apart by the shouldering. If you can’t tell.... try and let the history bias you. Cough? Fever? Think Croup.

Answer 108

epiglottitis retropharygeal abscess tonsis (adenoids) exudative tracheitis

Answer 109

Looks like a thumb ``` If the ordering suspects the diagnosis, do NOT bring this kid to x-ray. Have them do a portable. ```

Answer 110

``` Too wide ( > 6mm at C2, or > 22mm at C6) Next Step = CT don't forget to look in the mediastinum for “Danger Zone” extension. ```

Answer 111

Not seen till about 3-6 months, and not big till around 1-2 years. Too big when they encroach the airway

Answer 112

Linear Filling Defect It’s usually staph

Answer 113

The easiest way to do this is to just count ribs. More than 6 Anterior, or 8 Posterior as they intersect the diaphragm is too much. As a quick review, remember that the anterior ribs (grey) are the ones with a more sloping course as they move medially, where as the posterior ribs (black) have a horizontal course.

Answer 114

* Flattening o f the diaphragms | * Ribs take on a more horizontal appearance

Answer 115

A. Go to google images B. Search “Granular neonatal chest x-ray.'" Look at a bunch o f examples. Maybe even download a few of them for review. C. Search “Streaky Perihilar neonatal chest x-ray." Look at a bunch o f examples. Maybe even download a few o f them for review. D. Search "Ropy neonatal chest x-ray." Look at a bunch o f examples. Maybe even download a few o f them for review.

Answer 116

We are going to talk about the presence o f a pleural effusion as a discriminator. One pearl is to look fo r an accentuated (thick) minor fissure on the right. If you see that shit, kid probably has an effusion. Confirm by staring with fierce intensity at the lung bases to look for obliteration o f the costophrenic sulcus.

Answer 117

Alphabet - MNoP Meconium Aspiration Non GB Neonatal Pneumonia

Answer 118

SSD | Group B Pneumonia

Answer 119

This typically occurs secondary to stress (hypoxia), and is more common in term or postmature babies (the question stem could say “post term ” delivery). The pathophysiology is all secondary to chemical aspiration.

Answer 120

* The buzzword “ropy appearance” o f asymmetric lung densities * Hyperinflation with alternative areas o f atelectasis * Pneumothorax in 20-40% o f cases

Answer 121

How can it have hyperinflation?? Aren V the lungs fu l l o f sticky shit (literally) ??? The poop in the lungs act like miniature bal 1-valves (“floaters” 1 call them), causing air trapping - hence the increased lung volumes.

Answer 122

“Meconium Staining ” on the amniotic fluid is common (like 15% o f all births), but development o f “aspiration syndrome” is rare with only 5% o f those 15% actually have aspiration symptoms. Having said that, if the question header bothers to include “Green colored amniotic fluid” or “Meconium staining” in the question header they are giving you a major hint. Don't overthink a hint like this. I f they ask “What color was George Washington s white horse ? ” the answer is NOT brown.

Answer 123

The classic clinical scenario is a history o f c-section (vagina squeezes the fluid out of lungs normally). Other classic scenario histories include “diabetic mother” and/or “maternal sedation.” Findings are going to start at 6 hours, peak at one day, and be done by 3 days. You are going to see coarse interstitial marking and fluid in the fissures.

Answer 124

* Classic histories: C-Section, Maternal Sedation, Maternal Diabetes * Onset: Peaks at day 1, Resolved by Day 3 * Lung Volumes - Normal to Increased

Answer 125

This is also called hyaline membrane disease, or RDS. It’s a disease of pre-mature kids. The idea is that they are bom without surfactant (the stuff that makes your lungs stretchy and keeps alveolar surfaces open). It’s serious business and is the most common cause of death in premature newborns. You get low lung volumes and bilateral granular opacities (just like B-hcmolytic pneumonia). But, unlike B-hemolytic pneumonia you do NOT get pleural effusions. As a piece of useful clinical knowledge, a normal plain film at 6 hours excludes SDD.

Answer 126

They can spray this crap in the kid’s lungs, and it makes a huge difference (decreased death rate etc...). Lung volumes get better, and granular opacities will clear centrally after treatment. The post treatment look of bleb-like lucencies can mimic PIE.

Answer 127

* Increased Risk of Pulmonary Hemorrhage | * Increased Risk of PDA

Answer 128

This is the most common type of pneumonia in newborns. It’s acquired during exit of the dirty birth canal. Premature infants arc at greater risk relative to term infants. It has some different looks when compared to other pneumonias (why I discuss it separately).

Answer 129

* It often has low lung volumes (other pneumonias have high) * Granular Opacities is a buzzword (for this and SDD) * Often (25%) has pleural effusion (SDD will not) * LESS likely to have pleural effusion compared to the non Beta hemolytic version (25% vs 75%)

Answer 130

Lots of causes. Typical look is patchy, asymmetric pcrihilar densities, effusions, and hyperinflation. Will look similar to surfactant deficient disease but will be full term. Effusions are also much more likely (they arc rare in SDD).

Answer 131

Also called “persistent fetal circulation”. Normally, the high pulmonary pressures seen in utero (that cause blood to shunt around the lungs) decrease as soon as the baby takes his/her first breath. Dr. Goljan (Step 1 wizard) calls this a “miracle,” and used this basic physiology to deny evolution. When high pressures persist in the lungs it can be primary (the work of Satan), or secondary from hypoxia (meconium aspiration, pneumonia, etc...). The CXR is going to show the cause of the pulmonary HTN (pneumonia), rather than the HTN itself.

Answer 132

You Say Meconium Aspiration

Answer 133

You say Transient Tachypnea

Answer 134

You say Transient Tachypnea

Answer 135

You say RDS

Answer 136

* Meconium Aspiration * Transient Tachypnea * Non BH Neonatal Pneumonia

Answer 137

* Surfactant Deficiency (no pleural effusion) | * Beta-Hemolytic Pneumonia (gets pleural effusions)

Answer 138

When you have surfactant deficiency and they put you on a ventilator (which pulverizes your lungs with PEEP), you can end up with air escaping the alveoli and ending up in the interstitium and lymphatics. On CXR it looks like linear lucencies (buzzword). It’s a warning sign for impending Pneumothorax. Most cases o f PIE occur in the first week of time (bronchopulmonary dysplasia - which looks similar - occurs in patients older than 2-3 weeks). Surfactant therapy can also mimic PIE. The treatment is to switch ventilation methods and/or place them PIE side down.

Answer 139

* Consequence of ventilation * Usually occurs in the first week of life * Buzzword = Linear Lucencies * Warning Sign for Impending Pneumothorax * Treatment is to put the affected side down

Answer 140

A total zebra is the progression of PIE to a large cystic mass. The thing can even cause mediastinal mass effect.

Answer 141

This is the kid bom premature (with resulting surfactant deficiency), who ends up being tortured in ventilator purgatory. His/her tiny little lungs take a ferocious ass whipping from positive pressure ventilation and oxygen toxicity — “barotrauma” they call it. This beating essentially turns the lungs into scar, inhibiting their ability to grow correctly. That is why people call this “a disease of lung growth impairment.”

Answer 142

Prolonged ventilation in a tiny (<1000 grams), premature kid (<32 weeks)

Answer 143

Alternating regions of fibrosis (coarse reticular opacities), and hyper-aeration (cystic lucencies).

Answer 144

“Band like opacities”

Answer 145

PIE = First week of life, CLD = After 3 to 4 weeks' postnatal age

Answer 146

This can be primary or secondary. Secondary causes seem to lend themselves more readily to multiple choice questions. Secondary causes can be from decreased hemi-thoracic volume, decreased vascular supply, or decreased fluid. The most common is the decreased thoracic volume, typically from a space occupying mass such as a congenital diaphragmatic hernia (with bowel in the chest), but sometimes from a neuroblastoma or sequestration. Decreased fluid, refers to the Potter Sequence (no kidneys -> no pee -> no fluid -> hypoplastic lungs).

Answer 147

These are grouped into intralobar and extralobar with the distinction being which has a pleural covering. The venous drainage is different (intra to pulmonary veins, extra to systemic veins). You can NOT tell the difference radiographically. The practical difference is age of presentation; intralobar presents in adolescence or adulthood with recurrent pneumonias, extralobar presents in infancy with respiratory compromise.

Answer 148

Much more common (75%). Presents in adolescence or adulthood as recurrent pneumonias (bacteria migrates in from pores of Kohn). Most commonly in the left lower lobe posterior segment (2/3s). Uncommon in the upper lobes. In contradistinction from extralobar sequestration, it is rarely associated with other developmental abnormalities. Pathology books love to say “NO pleural cover ” - but you can’t see that shit on CT or MR.

Answer 149

more common presents in adolescence recurrent infections no pleural cover pulmonary venous drainage

Answer 150

Less common of the two (25%). Presents in infancy with respiratory compromise (primarily because of the associated anomalies - Congenital cystic adenomatoid malformation (CCAM), congenital diaphragmatic hernia, vertebral anomalies, congenital heart disease, pulmonary hypoplasia). It rarely gets infected since it has its own pleural covering. These are sometimes described as part of a bronchopulmonary foregut malformation, and may actually have (rarely) a patent channel to the stomach, or distal esophagus. Pathology books love to say “has a pleural cover ” - but you can’t see that shit on CT or MR.

Answer 151

less common presents in infancy associated congenital anomalies

Answer 152

I say recurrent pneumonia in same area, you say intralobar sequestration.

Answer 153

Typically an incidental finding. They are generally solitary and unilocular. They typically do NOT communicate with the airway, so if they have gas in them you should worry about infection.

Answer 154

As the name suggests it’s a malformation of adenomatoid stuff that replaces normal lung. Most of the time it only affects one lobe. There is no lobar preference (unlike CLE which favors the left upper lobe). There are cystic and solid types (type 1 cystic, type 3 solid, type 2 in the middle). There is a crop of knuckle heads who want to call these things CPAMs and have 5 types, which I ’m sure is evidence based and will really make an impact in the way these things are treated. CCAMs communicate with the airway, and therefore at least components of them can fill with air. Most of these things (like 90%) will spontaneously decrease in size in the third trimester. The treatment (at least in the USA) is to cut these things out, because of the iddy bitty theoretical risk of malignant transformation (pleuropulmonary blastoma, rhabdomyosarcoma).

Answer 155

Then it’s not a CCAM, it’s a Sequestration. — mumble to yourself “nice try assholes”

Answer 156

The idea behind this one is that you have bronchial pathology (maybe atresia depending on what you read), that leads to a ball-valve anomaly and progressive air trapping. On CXR, it looks like a lucent, hyper-expanded lobe.

Answer 157

* It’s not actually emphysema - just air trapping secondary to bronchial anomaly * It prefers the left upper lobe (40%) * Treatment is lobectomy

Answer 158

•The classic way this is shown in case conference or case books is with a series of CXRs. The first one has an opacity in the lung (the affected lung clears fluid slower than normal lung). The next x-ray will show the opacity resolved. The following x-ray will show it getting more and more lucent. Until it’s actually pushing the heart over.

Answer 159

Most commonly they are Bochdalek type. B is in the Back - they are typically posterior and to the left. The appearance on CXR is usually pretty obvious.

Answer 160

* Usually in the Back , and on the left (Bochdalek) * If it’s on the right - there is an association with GBS Pneumonia * Mortality Rate is related to the degree o f pulmonary Hypoplasia * Most have Congenital Heart Disease * Essentially all are malrotated

Answer 161

•One trick is to show the NG tube curving into the chest.

Answer 162

``` Think Congenital Lobar Emphysema (CLE) first But, remember CCAM has no lobar prevalence, so it can be anywhere ```

Answer 163

Think Sequestration First Congenital Diaphragmatic Hernia (CDHs) favors this side too

Answer 164

In all ages this is way more common than bacterial infection. Peribronchial edema is the buzzword for the CXR finding. “Dirty” or "Busy” Hilum. You also end up with debris and mucus in the airway which causes two things (1) hyperinflation and (2) subsegmental atelectasis. Respiratory Syncytial Virus (RSV) - This will cause the typical non-specific viral pattern as well. However, there is the classic testable predilection to cause a segmental or lobar atelectasis — particularly in the right upper lobe.

Answer 165

Kids get round pneumonia. They love to show this, and try to trick you into thinking it’s a mass. Younger than 8 you are thinking round pneumonia, round pneumonia, round pneumonia - with S. Pneumonia being the culprit. The PhD trivia is that these occur because you don’t have good collateral ventilation pathways. Round pneumonia is usually solitary, and likes the posterior lower lobes. Take home message: No CT to exclude cancer, just get a follow up x-ray.

Answer 166

It is best to think about this as a cousin or uncle of Round Pneumonia (they are in the same family). It is essentially the same thing except it is peripheral. The classic look is a round, pleural based “mass” in the apex of the lung. Similarly to the “round pneumonia” this is a transient finding and will resolve as the primary process improves.

Answer 167

Classic history is a parent giving their newborn a teaspoonful of olive oil daily to cultivate “a spirit o f bravado and manliness. ” Although this seems like a pretty solid plan, and I can’t fault their intentions - it’s more likely to result in chronic fat aspiration. Hot Sauce is probably a better option. Most people will tell you that bronchoalveolar lavage is considered the diagnostic method of choice. CXR nonspecific - it is just airspace opacities. CT is much more likely to be the modality used on the exam. The classic finding is low attenuation (-30 to -100 HU) within the consolidated areas reflecting fat content

Answer 168

The key concept is that it causes air trapping. The lung may look more lucent (from air trapping) on the affected side. You put the affected side down and it will remain lucent (from air trapping). Another random piece of trivia is that under fluoro the mediastinum will shift AWAY from the affected side on expiration.

Answer 169

This is the classic unilateral lucent lung. It typically occurs after a viral lung infection in childhood resulting in post infectious obliterative bronchiolitis. The size of the affected lobe is smaller than a normal lobe (it’s not hyper-expanded).

Answer 170

Perinatal HPV can cause these soft tissue masses within the airway and lungs. It’s also seen in adults who smoke. “Multiple lung nodules which demonstrate cavitation” is the classic scenario. Some testable trivia includes the 2% risk of squamous cell cancer, and that manipulation can lead to dissemination. The appearance of cysts and nodules can look like LCH (discussed more in the thoracic chapter), although the trachea is also involved.

Answer 171

Kids with sickle cell can get “Acute chest.” Acute chest actually occurs more in kids than adults (usually between age 2-4). This is the leading cause of death in sickle cell patients. Some people think the pathology is as such: you infarct a rib -> that hurts a lot, so you don’t breath deep -> atelectasis and infection. Others think you get pulmonary microvascular occlusion and infarction. Regardless, if you see opacities in the CXR of a kid with sickle cell, you should think of this.

Answer 172

So the sodium pump doesn’t work and | they end up with thick secretions and poor pulmonary clearance. The real damage is done by recurrent infections.

Answer 173

The motile part of the cilia doesn’t work. They can’t clear their lungs and get recurrent infections. These guys have lots of bronchiectasis just like CF. BUT, this time it’s lower lobe predominant (CF was upper lobe

Answer 174

A normal inspiratory CXR is meaningless. Don’t forget that the crayon / green bean is going to be radiolucent. You need expiratory films to elicit air trapping. Normally, the bottom lung is gonna turn white (move less air). If there is air trapping the bottom lung will stay black.

Answer 175

(how do you know it’s sickle cell?) •Kid with Big Heart •Kid with bone infarcts (look at the humeral heads) •Kid with H shaped vertebra (look on lateral)

Answer 176

•Bronchiectasis (begins cylindrical and progresses to varicoid) •It has an apical predominance (lower lobes are less affected) •Hyperinflation •They get Pulmonary Arterial Hypertension •Mucus plugging (finger in glove sign) •Men are infertile (vas deferens is missing)

Answer 177

• Fatty Replaced Pancreas on CT • Abdominal Films with Constipation ``` Biliary Cirrhosis (from blockage of intrahepatic bile ducts), and resulting portal HTN ```

Answer 178

•Bronchiectasis (lower lobes) •50% will have Kartageners (situs inversus). So, 50% will not •Men are infertile (sperm tails don’t work) •Women are sub-fertile (cilia needed to push eggs around)

Answer 179

``` CF: Upper Lobe Predominant - Brochiectasis Infertile - Men are Missing the Vas Deferens ``` ``` Primary Ciliary dyskinesia: Lower Lobe Predominant - Bronchiectasis Infertile - Men’s Sperm Don’t Swim For Shit ```

Answer 180

This is a primary intrathroacic malignancy. They can look a lot like CCAMs and even have different types (cystic, mixed, solid). These things are usually right sided, pleural based, and without chest wall invasion or calcifications. No rib invasion (helps distinguish it from the Askin / Ewing Sarcoma o f the Chest Wall - if they won’t tell you the age), No calcification. The more solid types can have mets to the brain and bones. The cystic type seem to occur more in kids less than a year old, and be more benign.

Answer 181

* Big Fucking Mass (B.F.M.) in the chest o f a 1-2 year old * Shouldn’t have an eaten-up rib (Askin tumors often do) * 10% o f the time they have a multilocular cystic nephroma.

Answer 182

A UVC passes from the umbilical vein to the left portal vein to the ductus venosus to a hepatic vein to the 1VC. You don’t want the thing to lodge in the portal vein because you can infarct the liver. The ideal spot is at the 1VC - Right Atrium junction.

Answer 183

UVC erosion into the liver.

Answer 184

A UAC passes from the umbilicus, down to the umbilical artery, into an iliac artery then to the aorta. Positioning counts, as the major risk factor is renal arterial thrombosis. You want to avoid the renal arteries by going high (T8-T10), or low (L3-L5)

Answer 185

* It goes down first * It should be placed either high (T8-T10) or low (L3-L5) * Omphalocele is a contraindication

Answer 186

down then up

Answer 187

straight up

Answer 188

Neonatologists primarily use this device to torture sick babies - hopefully into revealing the various government secrets they have stolen, or the location of their organization’s underground lair. “Enhanced interrogation” or “temporizing measure o f last resort,” they call it - to get around the Geneva Conventions.

Answer 189

Alternatively, it can be used as a last resort in neonatal sepsis, severe SSD, and meconium aspiration. Actually, in cases of meconium aspiration ECMO actually does work (sometimes).

Answer 190

There are two main types: (1) Veno-Arterial “V-A”, and (2) Veno-Venous “V-V” In both cases deoxygenated blood is removed from the right atrium and pumped into a box (artificial lung) to get infused with oxygen. The difference is in how it is returned.

Answer 191

In V-A, blood goes back to the Aorta (you can see why this would help rest the left ventricle). The catheter is usually placed at the origin o f the innominate or “overlying the arch.”

Answer 192

In V-V blood goes right back into the right atrium. In this situation even if the lungs were totally clogged with shit (meconium aspiration) and no oxygen exchange was happening it wouldn't matter because the blood that is being pumped (RA -> RV -> Pulmonary Artery) already has oxygen in it.

Answer 193

``` Catheter Position: RA + Aorta (near the origin of the innominate artery) ``` ``` Catheter Position: RA + Aorta (near the origin of the innominate artery) ```

Answer 194

Catheter Position: RA+ RA “Dual Lumen” Lung Support

Answer 195

Mechanism is variable depending on who you asked. The way 1 understand it is that the airway pressure suddenly drops off causing atelectasis, plus you have a change in the circulation pattern that now mimics the fetal physiology (mom = artificial lung). Resulting oxygen tension changes lead to an edema like pattern. A multiple choice trick could be to try and make you say it is worsening airspace disease, or reflects the severity of the lung injury. Don’t fall for that. It’s an expected finding.

Answer 196

I mentioned on the prior page that they typically ligate the carotid when they place the Arterial catheter. No surprise that they will be at increased risk for neurologic ischemic complications as a result.

Answer 197

The combination of anticoagulation (necessary in ECMO) and being sick as stink puts these kids at super high risk for head bleeds. This is why they will get screened for germinal matrix hemorrhage prior to being placed on ECMO and then routinely screened with head ultrasounds (expertly read by the second year resident on call).

Answer 198

I think there are two likely ways a multiple choice question could be structured related to ECMO catheter position. The first would be to show you a series of daily radiographs with the latest one demonstrating migration of one or both catheters. Thats the easy way to do it. The sneaky way would be to show you the catheter with the lucent distal end and the dot marker on the tip. This would be particularly evil as there are tons of different catheter brands and looks, but if I was going to try and trick you, this would be the way that I would do it. In this case, the venous catheter looks falsely high if judged by the lucent tip, but that round metallic marker (near the black arrow) shows the tip in the RA. The arterial catheter “overlies the region of the aortic arch,” which is normal.

Answer 199

Normal Thymus Thymic Rebound Lymphoma: Germ Cell Tumor (GCT):

Answer 200

This is the most common mediastinal “mass.” It’s terribly embarrassing to call a normal thymus a mass, but it can actually be tricky sometimes. It can be pretty big in kids less than 5 (especially in infants). Triangular shape of the thymus is sometimes called the “sail sign.” Not to be confused with the other 20 sail signs in various parts of the body, or the spinnaker sail sign, which is when pneumomediastinum lifts up the thymus.

Answer 201

In times of acute stress (pneumonia, radiation, chemotherapy, bums), the thymus will shrink. In the recovery phase it will rebound back to normal, and sometimes larger than before. During this rebound it can be PET avid

Answer 202

This is the most common abnormal mediastinal mass in children (older children and teenagers). Lymphoma vs Thymus can be tricky. Thymus is more in kids under 10, Lymphoma is seen more in kids over 10. When you get around age 10, you need to look for cervical lymph nodes to make you think lymphoma. If you see calcification, and the lesion has NOT been treated you may be dealing with a teratoma. Calcification is uncommon in an untreated lymphoma.

Answer 203

Compression o f SVC, Compression o f Pulmonary> Veins, Pericardial Effusion, Airway Compression.

Answer 204

can appear very similar to Lymphoma (soft tissue mass in the anterior mediastinum). In this scenario, most people will tell you that Lymphoma can NOT be differentiated from Leukemia on imaging alone.

Answer 205

On imaging, this is a large anterior mediastinal mass arising from or at least next to the thymus. It comes in three main flavors,

Answer 206

Mostly Cystic, with fat and calcium

Answer 207

Bulky and Lobulated. “Straddles the midline ”

Answer 208

Big and Ugly - Hemorrhage | and Necrosis. Can get crazy and invade the lung.

Answer 209

``` • Abnormal Size for patients Age (really big in a 15 year old) • Heterogenous appearance • Calcification • Compression of airway or vascular structure ```

Answer 210

lymphadenopathy duplications cysts

Answer 211

``` Middle mediastinal lymphadenopathy is most often from granulomatous disease (TB or Fungal), or from lymphoma. ```

Answer 212

Cause: EBV (-90%), CMV (-10%) Classic Scenario: Adolescent with hilar adenopathy, splenomegaly, and fatigue. Trivia: Rash after Antibiotics (Amoxicillin) Trivia: “No sports for 3 weeks” - to avoid splenic injury

Answer 213

Distribution of nodes in usually unilateral, right hilar, right paratracheal Lower and Middle lobe consolidation is common

Answer 214

Midwest and Southeast United States Most have normal CXRs Can have hilar adenopathy

Answer 215

Southwest United States Usually looks like consolidation and nodules Can have hilar adenopathy

Answer 216

``` Often cited as the “most common” anterior mediastinal mass in a kid. Hilar involvement (usually bilateral) is more common with Hodgkin ```

Answer 217

Uncommon in children — it usually presents in the early-mid 20s. Case reports of early onset Sarcoid exist.

Answer 218

These fall into three categories (a) bronchogenic, (b) enteric, (c) neuroenteric. The neuroenterics are traditionally posterior mediastinal.

Answer 219

water attenuation - close to the trachea or bronchus. Trivia: • Tend to be middle mediastinal (70%), the rest are in the hilum • Typically filled with mucus or fluid

Answer 220

water attenuation close to the esophagus (lower in the mediastinum). Trivia: • Abutment o f the esophagus is the key finding. • Can communicate with the lumen of the esophagus - and have air/fluid levels. • Usually on the right, involving the distal esophagus • Can be middle or posterior mediastinal in location • Second most common G1 lumen duplication cyst (distal ileum #1)

Answer 221

Neuroblastoma Ewing Sarcoma Askin Tumor (Primitive Neuroectodermal tumor of the chest wall): Neuroenteric Cyst Extramedullary Hematopoiesis

Answer 222

This is the most common posterior mediastinal mass in a child under 2. This is discussed in complete detail in the GU PEDs section. I’ll just mention that compared to abdominal neuroblastoma, thoracic neuroblastoma has a better outcome. It may involve the ribs and vertebral bodies. Also, remember that Wilms usually mets (more than neuroblastoma) to the lungs, so if it’s in the lungs don’t forget about Wilms.

Answer 223

neuroblastoma > ganglioneruoblastoma > ganglioneuroma

Answer 224

• Less Aggressive • More Circumscribed (less invasive) • Less Likely to have Calcifications (although they still can) • Pound in Older Children These tumors can NOT be differentiated with imaging alone (but that doesn't mean someone can’t write a multiple choice question asking you to try).

Answer 225

This is now considered part of the Ewing Sarcoma spectrum, and is sometimes called an Ewing sarcoma of the chest wall. They tend to displace adjacent structures rather than invade early on (when they get big they can invade). They look heterogenous, and the solid parts will enhance.

Answer 226

By convention these are associated with vertebral anomalies (think scoliosis, hemivertebrae, butterfly vertebrae, split cord, etc..) - think cyst protruding out of an unsealed canal / defect. The cyst does NOT communicate with CSF, is well demarcated, and is water density. Favor the lower cervical and thoracic regions.

Answer 227

This occurs in patients with myeloproliferative disorders or bone marrow infdtration (including sickle cell). Usually, this manifests as a big liver and big spleen. However, in a minority of cases you can get soft tissue density around the spine (paraspinal masses), which are bilateral, smooth, and sharply delineated.

Answer 228

This occurs in patients with myeloproliferative disorders or bone marrow infdtration (including sickle cell). Usually, this manifests as a big liver and big spleen. However, in a minority of cases you can get soft tissue density around the spine (paraspinal masses), which are bilateral, smooth, and sharply delineated.

Answer 229

In a kid just assume it’s Hodgkins (which means it’s gonna involve the thymus). Why assume Hodgkins ? Hodgkins is 4x more common than NHL. Hodgkins involves the thymus 90% of the time.

Answer 230

My main move is to go age. Under 10 = Thymus, Over 10 = Lymphoma.

Answer 231

If the test writer is headed in this direction they MUST either (a) bias you with a history saying stuff like “got off chemo” or “got off corticosteroids” or (b) show you a series of axial CTs with the thing growing and maintaining normal morphology. I think “a” is much more likely.

Answer 232

In general just think morphology / density: • Soft Tissue - Kinda Homogenous = Think Lymphoma or Hyperplasia • Fat = Germ Cell Tumor (Why God!? Why Klinefelters!?) • Water = Congenital Stuff - Think Lymphangiomas

Answer 233

Think malignant, | Think neuroblastoma.

Answer 234

Think benign. If it’s a round mass- Think about Ganglioneuromas & Neurofibromas If it’s cystic (and there is scoliosis) think Neuroenteric Cyst If they show you coarse bone trabeculation - with an adjacent mass (or a history of anemia) - Think Extramedullary Hematopoiesis

Answer 235

``` “Big Fucking Mass” If you see a B.F.M. in the chest o f a kid, you basically have two choices: (1) Askin Tumor (PNET / Ewings) - * AGE 10+, look for an eaten up rib. (2) Pleuropulmonary Blastoma *AGE is typically less than 2. ```

Answer 236

This can occur in multiple subtypes, with the classic ways of showing it being a frontal CXR with an NG tube stopped in the upper neck, or a fluoro study (shown lateral) with a blind ending sac or communication with the tracheal tree. There are 5 main subtypes, only 3 (shown above) are worth knowing (being familiar with) for the purpose of the exam.

Answer 237

•Diagnosis is made with a Fluoro swallow exam •Most Important Thing To Know are the VACTERL associations (more on this later) • The most common subtype is the N-Type (blind ended esophagus, with distal esophagus hooked up to trachea • Excessive Air in the Stomach = H type (can also be with N type) • No Air in the Stomach = Esophageal Atresia • The presence of a right arch (4%) must be described prior to surgery (changes the approach).

Answer 238

Fake out for TE fistula is simple aspiration. Look for the presence or absence of laryngeal penetration to tell them apart (if shown a dynamic Fluoro swallow exam).

Answer 239

This is extremely high yield. VACTERL is a way of remembering that certain associations are seen more commonly when together (when you see one, look for the others).

Answer 240

``` V - Vertebral Anomalies (37%) A - Anal (imperforate anus) (63%) C - Cardiac (77%) TE - Tracheoesophageal Fistula or Esophageal Atresia (40%) R - Renal (72%) L - Limb (radial ray) - 58% ```

Answer 241

VACTERL association is diagnosed when 3 or more of the defined anomalies affect a patient. Therefore, keep investigating when 1 -2 of these anomalies are found. The heart and kidneys are the most commonly affected organs in this

Answer 242

If both limbs are involved, then both kidneys tend to be involved. If one limb is involved, then one kidney tends to be involved

Answer 243

Around 30% of kids with a repaired esophageal atresia will end up with a focal anastomotic stricture. Strictures can also be seen with caustic ingestion (dishwashi

Answer 244

Kids love to stick things in their mouths (noses and ears). This can cause a lot of problems including direct compression o f the airway, perforation, or even fistula to the trachea. Stuff stuck in the esophagus needs to be removed.

Answer 245

The esophagus is a dirty sock, it flexes to accommodate that big piece of steak you didn’t even bother to chew. The trachea is rigid, like that math teacher I had in high school (who hated music... and colors), but unlike the math teacher it has a flexible membrane in the back. The point of me mentioning this is to help you problem solve a “where is the coin ” type question. The esophagus will accommodate the coin so it can be turned in any direction. The trachea is rigid and will force the coin to rotate into the posterior membrane — so it will be skinny in the AP direction.

Answer 246

One magnet is ok. Two or more magnets is a problem. The reason is that they can attract each other across intestinal walls leading to obstruction, necrosis, perforation, and a law suit. Surgical Consult NOT an MRI (dumbass)

Answer 247

Less of a problem relative to other types of batteries, but can cause serious problems if you need them for the DVR control because Game of Thrones season 7 is on (spoiler it sucked). Serial plain film exams. Remove if they stay in the stomach for more than 2 days

Answer 248

``` They look like coins, except they have two rings. The literature is not clear, but it appears that modern batteries rarely leak (leaking is bad - caustic chemicals, heavy metals etc..). ``` Stuck in the esophagus = big problem, gotta get them out within 2 hours. Stuck in the stomach = problem, gotta get them out within 4 days.

Answer 249

Copper Pennies are relatively safe. Make sure it is not a disc battery (coins have one order ring, disc battery has two). ``` Remove if: Retention in the esophagus for more than 24 hours or Stomach for more than 28 days. ```

Answer 250

Those minted after 1982 contain mostly Zinc which when combined with stomach acid can cause gastric ulcerations, and if absorbed in great enough quantity can cause zinc toxicosis (which is mainly pancreatic dysfunction / pancreatitis). The ulcers are the more likely thing to happen, so just remember that. So how the hell can you tell the date of a penny that is swallowed? Either (a) the question stem will have to say something like - “2 year old child playing with father’s collection of 1984 pennies” , or the more likely (b) showing you the penny with characteristic radiolucent holes - from erosion. remove rom stomach

Answer 251

Usually Lead Paint Dust - or Any object with lead paint. Bad because the gastric acid leads to immediate absorption. Remove immediately from stomach. Distal passable must be confirmed.

Answer 252

Esophagus = Remove Immediately Stomach = Remove Immediately Post Pylorus = Follow vs Surgery — if it does perforate the small bowel it will be at the IC valve

Answer 253

• The only variant that goes between the esophagus and the trachea. • Classic question is that this is associated with tracheal stenosis (which is actually primary and not secondary to compression). • High association with other cardiopulmonary and systemic anomalies: hypoplastic right lung, horseshoe lung, TE-fistula, imperforate anus, and complete tracheal rings. • Treatment is controversial but typically involves surgical repositioning o f the artery

Answer 254

most common symptomatic ascular ring anomaly impressions on the anterior aorta and posterior trachea

Answer 255

• Most Common Aortic Arch Anomaly — not necessarily symptomatic. • “Dysphagia Lusoria” - fancy Latin speak (therefore high yield) for trouble swallowing in the setting of this variant anatomy • “Diverticulum of Kommerell” pouch like aneurysmal dilatation of the proximal portion o f an aberrant right subclavian artery

Answer 256

Midgut Volvulus / Malrotation Duodenal Atresia Duodenal Web annular pancrease jejunal atresia

Answer 257

hirschprung disease meconium plug syndrome ileal atresia meconium ileus anal atresia/colonic atresia

Answer 258

Gastric (antral or pyloric) atresia.

Answer 259

Duodenal Atresia

Answer 260

``` • 30% have Downs • 40% have polyhydramnios and are premature • The“singlc atresia” - cannulation error • On multiple choice test the “double bubble” can be shown on 3rd trimester OB ultrasound, plain film, or on MRI. ```

Answer 261

Jejunal Atresia. When you call jejunal atresia, you often prompt search for additional atresias (colonic). Just remember !^ ,' P that jejunal atresia is secondary to a vascular insult during development.

Answer 262

vascular error

Answer 263

Can mean nothing (lotta air swallowing). If the clinical history is bilious vomiting , this is ominous and can be midgut volvulus (surgical emergency). Next test would be emergent Upper GI.

Answer 264

Seeing distal gas excludes duodenal atresia. The DDx is a duodenal web, duodenal stenosis, or midgut volvulus. Next step would be upper GI.

Answer 265

Suggestive o f a low obstruction (ileum or colon). Next step is contrast enema. If the contrast enema is normal you need to follow with upper GI (to exclude an atypical look for midgut volvulus).

Answer 266

“Sick Belly” - Can be seen with proximal or distal obstruction. Will need Upper GI and contrast enema.

Answer 267

Normally, the developmental rotation o f the gut places the ligament of Trietz to the left o f the spine (at the level o f the duodenal bulb). If mother nature fucks up and this doesn’t happen, you end up with the duodenum to the right o f the midline (spine). These patients are at increased risk for midgut volvulus and internal hernias. If you see the appearance o f malrotation and the clinical history is bilious vomiting, then you must suspect midgut volvulus

Answer 268

•Associated with Heterotaxy Syndromes. Associated with Omphaloceles. •Classically shown as the SMA to the right of the SMV (on US or CT). •False Positive on UGI - Distal Bowel Obstruction displacing the duodenum (because of ligamentous laxity).

Answer 269

You Say Hypertrophic Pyloric Stenosis next step ultrasound

Answer 270

You Say Mid Gut Volvulus (till proven otherwise) | Next Step ? Upper GI

Answer 271

This is diagnostic of midgut volvulus (surgical emergency). The appearance is an Aunt Minnie.

Answer 272

In older children (or even adults) obstruction in the setting of malrotation will present as intermittent episodes o f spontaneous duodenal obstruction. The cause is not midgut volvulus (a surgical emergency) but rather kinking from Ladd’s Bands.

Answer 273

We are talking about a fibrous stalk of peritoneal tissues that fixes the cecum to the abdominal wall, and can obstruct the duodenum.

Answer 274

Strongly associated with midgut volvulus. If you were thinking duodenal atresia, look for distal air (any will do) to exclude that thought.

Answer 275

``` Procedure to prevent midgut volvulus. Traditionally, the Ladd’s Bands are divided, and the appendix is taken out. The small bowel ends up on the right, and the large bowel ends up on the left. They are fixed in place by adhesions (just by opening the abdomen). It is still possible to develop volvulus post Ladd’s (but it’s rare - 2-5%). ```

Answer 276

* Divide the Adhesive Ladds Bands * Widen the Mesentery to a safe distance * Take out the appendix (bill extra for that)

Answer 277

If the kid is vomiting this might be from extrinsic narrowing (Ladd band, annular pancreas), or intrinsic (duodenal web, duodenal stenosis). You can’t tell.

Answer 278

Thickening of the gastric pyloric musculature, which results in progressive obstruction. Step 1 buzzword is “non-bilious vomiting.” Here is the most likely multiple choice trick; this does NOT occur at birth or after 3 months. There is a specific age range o f 2-12 weeks (peak at 3-6 weeks). Criteria is 4mm and 14mm (4mm single wall, 14mm length). The primary differential is pylorospasm (which will relax during exam). The most common pitfall during the exam is gastric over distention, which can lead to displacement o f the antrum and pylorus - leading to false negative. False positive can result from off axis measurement. The phenomenon of “paradoxical aciduria” has been described, and is a common buzzword.

Answer 279

``` The greater curvature flips over the lesser curvature (rotation along the long axis). This is seen in old ladies with paraesophageal hernias. ```

Answer 280

``` Twisting over the mesentery (rotation along short axis). The antrum flips near the GE junction. Can cause ischemia and needs to be fixed. Additionally this type causes obstruction. This type is more common in kids. ```

Answer 281

This is best thought of as “almost duodenal atresia. ” The reason I say that is, just like duodenal atresia, this occurs from a failure to canalize, but instead o f a total failure of canalization (like duodenal atresia) this bowel is only partially canalized, leaving behind a potentially obstructive web.

Answer 282

``` - Because the web is distal to ampulla of Vater - you get bile-stained emesis - Associated with malrotation and Downs syndrome - The “wind sock” defonnity is seen more in older kids - where the web-like diaphragm has gotten stretched. ```

Answer 283

Essentially an embryologic screw up (failure o f ventral bud to rotate with the duodenum), that results in encasement o f the duodenum. In Kids = Think Duodenal Obstruction In Adults = Think Pancreatitis

Answer 284

Look for pancreatic tissue (same enhancement as the nearby normal pancreas) encircling the descending duodenum.

Answer 285

``` Look for an extrinsic narrowing of the duodenum. Obviously this is non-specific (typical barium - voodoo), use the location and clinical history to bias yourself. ```

Answer 286

You basically have 4 choices: Normal, | Short Microcolon, Long Microcolon, and a Caliber Change from micro to normal.

Answer 287

• Think about Colonic Atresia

Answer 288

ONLY in patients with CF. The pathology is the result of thick sticky meconium causing obstruction o f the distal ileum. Contrast will reach ileal loops, and demonstrate multiple filling defects (meconium). This can be addressed with an enema.

Answer 289

This is the result o f intrauterine vascular insult. Contrast will NOT reach ileal loops. This needs surgery.

Answer 290

This is a transient functional colonic obstruction, that is self limited and relieved by contrast enema. *Most Testable Fact: It is NOT associated with CF. *2nd Most Testable Fact: It is seen in infants of diabetic mothers 01 if mom received magnesium sulfate for eclampsia

Answer 291

Failure of the ganglion cells to migrate and innervate the distal colon. Affected portions o f the colon are small in caliber, whereas the normally innervated colon appears dilated.

Answer 292

* It’s 4:1 more common in boys. * 10% association with Downs. * Diagnosis is made by rectal biopsy.

Answer 293

-Enema - Rectum smaller than the Sigmoid “Recto-sigmoid ratio < 1” -Enema - Rectum with “sawtooth pattern” Represents bowel spasm

Answer 294

``` (1) Newborn who fails to have BM > 48 hours (or classically > 72 hours) (2) “Forceful passage o f meconium after rectal exam” (3) One month old who shows up “sick as stink” with NEC bowel ```

Answer 295

This is a super rare variant o f Hirschsprungs, and can mimic microcolon. The piece of commonly asked trivia is that it can also involve the terminal ileum.

Answer 296

This is a potential complication o f bowel atresia or meconium ileus. It has a very characteristic look. It’s a calcified mass in the mid abdomen , traditionally shown on plain film. It is the result of a sterile peritoneal reaction to an in-utero bowel perforation. Usually, the perforation seals off prior to birth and there is no leak.

Answer 297

Imperforate or Ectopic Anus

Answer 298

you say “VACTERL ” | you say “Screening US fo r tethered cord”

Answer 299

Classic D D x “A IM ” - Appendicitis, Adhesions Inguinal Hernia, Intussusception Midgut Volvulus, Meckels

Answer 300

In children older than 4 this is the most common cause for bowel obstruction. I f they show this in the PEDs section it’s most likely to be on ultrasound. In that case you can expect a blindending tube, non-compressible, and bigger than 6 mm.

Answer 301

Big points are that indirect hernias are more common in kids, they are lateral to the inferior epigastric, and incarceration is the most common complication. Umbilical hernias are common in kids, but rarely incarcerate. Trivia to know: This is the most common cause o f obstruction in boy 1 month - 1 year

Answer 302

The age range is 3 months - 3 years, before or after that you should think o f lead points (90% between 3 months and 3 years d o n ’t have lead points). The normal mechanism is forward peristalsis resulting in invagination o f proximal bowel (the intussusceptum) into lumen o f the distal bowel (the intussuscipiens). They have to be bigger than 2.5 cm to ma tte r (in most cases- these are enterocolic), those that are less than 2.0 cm are usually small bowel-small bowel and may reduce spontaneously within minutes. Just like an appendix, in the peds section, I would anticipate this shown on ultrasound as either the target sign or pseudo-kidney.

Answer 303

exam) • Recurrence: Usually within 72 hours • Success Rates - 80-90% with air (Henoch-Schonlein purpura has a reduced success rate) • Risk o f Perforation - 0.5% • Air causes less peritonitis (spillage o f fecal material) than barium • Pressure should NOT exceed 120 mniHg • Needle decompression would be the next step if they perforate and get tension pneumoperitoneum.

Answer 304

(1) what is it ?- these should be straight forward as targets or pseudo kidneys, (2) lead points - stu ff like HSP (vasculitis), Meckle diverticulum, enteric duplication cysts, and (3) reduction trivia.

Answer 305

This is a congenital diverticulum o f the distal ileum. A piece o f total trivia is that it is a persistent piece o f the omphalomesenteric duct. Step 1 style, “rule o f 2s” occurs in 2% o f the population, has 2 types o f heterotopic mucosa (gastric and pancreatic), located 2 feet from the IC valve, it’s usually 2 inches long (and 2 cm in diameter), and usually has symptoms before the child is 2. I f it has gastric mucosa (the ones that bleed typically do) it will take up Tc-Pertechnetate ju s t like the stomach (hence the Meckel’s scan).

Answer 306

* Can get diverticulitis in the Meckels (mimic appendicitis) * GI Bleed from Gastric Mucosa (causes 30% o f symptomatic cases) * Can be a lead point for intussusception (seen with inverted diverticulum) * Can Cause Obstruction

Answer 307

Extra-abominal evisceration of neonatal bowel (sometimes stomach and liver) through a paraumbilical wall defect.

Answer 308

* It does NOT have a surrounding membrane (omphalocele docs) * It’s always on the RIGHT side. * Associated anomalies are rare (unlike omphalocele). * Maternal Serum AFP will be elevated (higher than that of omphalocele) * Outcome is usually good * For some reason they get bad reflux after repair. * Associated with intestinal atresias.

Answer 309

Congenital midlinc defect, with herniation of gut at the base of the umbilical cord.

Answer 310

•It DOES have a surrounding membrane (gastroschisis does not) •Associated anomalies are common (unlike gastroschisis) •Trisomy 18 is the most common associated chromosomal anomaly •Other associations: Cardiac (50%), Other GI, CNS, GU, Turners, Klinefelters, Beckwith-Wiedemann, Pentalogy of Cantrell •Outcomes are not that good, because of associated syndromes. •Umbilical Cord Cysts (Allantoic Cysts) are associated

Answer 311

1. Omphalocoele 2. Ectopia Cordis (abnormal location o f heart) 3. Diaphragmatic Defect 4. Pericardial Defect or Sternal Cleft 5. Cardiovascular malformations

Answer 312

Herniated bowel loop through the ventral body wall NOT surrounded by a membrane Umbilicus is Normal - positioned to the left o f the defect (defect is on the right) defect is on the right asociated anomalies are rare associated with intestinal atresias cause: probably environment which explains an association with bowel atresia

Answer 313

Herniated bowel loop through the ventral body wall Surrounded by a membrane (peritoneum) Umbilicus contains herniated b ow e l, and therefore is NOT normal defect is midline High A ssociation with Cardiac (50%) defects and Chromosomal Abnormalities Multiple Syndromic Associations including Turners, Klinefelters, Beckwith-Wiedemann, and the Legendary Pentalogy o f Cantrell Cause: Probably Genetic - which explains the associations with the various syndromes

Answer 314

Classic injury from bicycle handlebars (or child abuse!. You can also see this as a complication from endoscopy. You could be shown retroperitoneal gas as a way to suggest perforation.

Answer 315

These are developmental anomalies (failure to canalize). They don’t have to communicate with the GI lumen but can. They are most commonly in the ileal region (40%). They have been known to cause in utero bowel obstruction / perforation.

Answer 316

Strategy: A common way to show this is a cyst in the abdomen (on ultrasound). If you have a random cyst in the abdomen you need to ask yourself - “does this have gut signature? ” • Cyst with Gut Signature = Enteric Duplication Cyst • Cyst without Gut Signature = Omental Cyst • WTF is “Gut Signature ?” - It’s alternating bands of hyper and hypo echoic signal - supposedly representing different layers of bowel.

Answer 317

30% of the time they are associated with vertebral anomalies.

Answer 318

This is a cause of bowel obstruction in an older kid (20 year old) with cystic fibrosis. This is sometimes called the “meconium ileus equivalent, ” because you end up with a distal obstruction (as the name implies) secondary to dried up thick stool. It more commonly involves the ileum / right colon. Kids who get this, are the ones who aren’t compliant with their pancreatic enzymes.

Answer 319

Self-limiting, usually viral inflammatory condition of mesenteric lymph nodes. It is a classic clinical mimic of appendicitis. The finding is a cluster of large right lower quadrant lymph nodes.

Answer 320

This is bad news. The general thinking is that you have an immature bowel mucosa (from being premature or having a heart problem), and you get translocated bugs through this immature bowel. It’s best thought of as a combination of ischemic and infective pathology.

Answer 321

* Premature Kids (90% within the first 10 days of life) * Low Birth Weight Kids ( < 1500 grams) * Cardiac Patients (sometimes occult) - they can be full term * Kids who had perinatal asphyxia * Hirschsprung Kids that go home and come back - they present around month 1.

Answer 322

• Pneumatosis - most definitive finding; Look for Portal Venous Gas Next • Focal Dilated Bowel (especially in the right lower quadrant) - the terminal ileum / right colon is the region most affected by NEC • Featureless small bowel , with separation (suggesting edema). • Unchanging bowel gas pattern — this would be a dirty trick - showin

Answer 323

* First question - has the kid been feed? No food = No poop. | * Second question - is it staying still? Poop will move, Pneumatosis will stay still.

Answer 324

Use of maternal breast milk is the only parameter associated with decreased incidence of NEC.

Answer 325

The pancreas is nearly always (90%) with CF patients. Inspissated secretions cause proximal duct obstruction leading to the two main changes in CF: (1) Fibrosis (decreased T1 and T2 signal) and the more common one (2) fatty replacement (increased Tl). Patients with CF diagnosed as adults tend to have more pancreas problems than those diagnosed as children. Those with residual pancreatic exocrine function can have bouts of recurrent acute pancreatitis. Small (l-3mm) pancreatic cysts are common.

Answer 326

* Complete fatty replacement is the most common imaging finding in adult CF * Enlarged with fatty replacement = lipomatous pseudohypertrophy of the pancreas. * Fibrosing Colonopathy: Thick walled right colon as a complication of enzyme replacement therapy

Answer 327

The 2nd most common cause of pancreatic insufficiency in kids (CF #1). Basically, it’s a kid with diarrhea, short stature, and eczema. Will also cause lipomatous pseudohypertrophy of the pancreas.

Answer 328

You only have a ventral bud (the dorsal bud forgets to form). Since the dorsal buds makes the tail, the appearance is that of a pancreas without a tail. All you need to know is that (1) this sets you up for diabetes (most o f your beta cells are in the tail), and (2) it’s associated with polysplenia.

Answer 329

The most common cause of pancreatitis in peds is trauma (seat belt).

Answer 330

Another critical point to make is that non-accidental trauma can present as pancreatitis. If the kid isn’t old enough to ride a bike (handle bar injury) or didn’t have a car wreck (seat belt injury) you need to think NAT.

Answer 331

Even at a large pediatric hospital its uncommon to see more | than 1-2 of these a year. Obviously, they are still fair game for multiple choice

Answer 332

The most common pediatric solid tumor. It’s found in female adolescents (usually asian, or black). The outcomes are pretty good after surgical resection. If you get shown a case in the peds setting this is probably it.

Answer 333

Age 1 = Pancreatoblastoma Age 6 = Adenocarcinoma Age 15 = SPEN

Answer 334

Infantile Hepatic Hemangioma Hepatoblastoma Mesenchymal Hamartoma

Answer 335

Often 1. Associated with high output CHF, this is classically shown as a large heart on CXR plus a mass in the liver. The aorta above the hepatic branches o f the celiac is often enlarged relative to the aorta below the celiac because o f differential flow. Skin hemangiomas are present in 50%. Endothelial growth factor is elevated. These can be associated with Kasabach-Merritt Syndrome (the platelet eater).

Answer 336

Actually well. They tend to spontaneously involute without therapy over months-years - as they progressively calcify.

Answer 337

Most common primary liver tumor o f childhood (< 5). The big thing to know is that it’s associated with a bunch o f syndromes - mainly hemi-hypertrophy, Wilms, Beckwith-Weidemann crowd. Prematurity is a risk factor. This is usually a well circumscribed solitary right sided mass, that may extend into the portal veins, hepatic veins, and IVC. Calcifications are present 50% o f the time. AFP is elevated. Another piece of trivia is the hepatoblastoma may cause a precocious puberty from making bHCG.

Answer 338

(1) Associated with Wilms, (2) AFP, (3) Precocious Puberty

Answer 339

This is the predominately cystic mass (or multiple cysts), sometimes called a “developmental anomaly.” Because it’s a “developmental anomaly” it shouldn’t surprise you that the AFP is negative. Calcifications are UNCOMMON. What is common is a large portal vein branch feeding the tumor.

Answer 340

HCC Fibrolamellar Subtype Undifferentiated Embryonal Sarcoma

Answer 341

``` This is actually the second most common liver cancer in kids. You’ll see them in kids with cirrhosis (biliary atresia, Fanconi syndrome, glycogen storage disease). AFP will be elevated. ```

Answer 342

This is typically seen in younger patients (<35) without cirrhosis and a normal AFP. The buzzword is central scar. The scar is similar to the one seen in FNH with a few differences. This scar does NOT enhance, and is T2 dark (the FNH scar is T2 bright). As a point of trivia, this tumor is Gallium avid. This tumor calcifies more often than conventional HCC.

Answer 343

This is the pissed off cousin of the mesenchymal hamartoma. It’s also cystic, but the mass is much more aggressive. It will be a hypodense mass with septations and fibrous pseudocapsule. This mass has been known to rupture.

Answer 344

Mets: Think about Wilms tumor or Neuroblastoma Now. there are several other entities that can occur in the liver o f young children / teenagers including; Hepatic Adenoma, Hemangiomas, Focal Nodular Hyperplasia, and Angio Sarcoma. The bulk o f these are discussed in greater detail in the adult GI chapter.

Answer 345

are congenital dilations of the bile ducts -classified into 5 types by some dude named Todani. The high yield trivia is type 1 is focal dilation o f the CBD and is by far the most common. Type 2 and 3 are super rare. Type 2 is basically a diverticulum o f the bile duct. Type 3 is a “choledochocele.” Type 4 is both intra and extra hepatic. Type 5 is Caroli’s, and is intrahepatic only. I’ll hit this again in the GI chapter.

Answer 346

is an AR disease associated with polycystic kidney disease and medullary sponge kidney. The hallmark is intrahepatic duct dilation, that is large and secular. Buzzword is “central dot sign” which corresponds to the portal vein surrounded by dilated bile ducts.

Answer 347

This will be discussed in greater detail in the renal section, but kids with AR polycystic kidney disease will have cysts in the kidneys, and variable degrees of fibrosis in the liver. The degree o f fibrosis is actually the opposite of cystic formation in the kidneys (bad kidneys ok liver, ok kidneys bad liver).

Answer 348

Autosomal dominant disorder characterized by multiple AVMs in the liver and lungs. It leads to cirrhosis, and a massively dilated hepatic artery. The lung AVMs set you up for brain abscess.

Answer 349

If you have prolonged newborn jaundice (> 2 weeks) you should think about two things (1) neonatal hepatitis, and (2) Biliary Atresia. It’s critical to get this diagnosis right because they need corrective surgery (Kasai Procedure) prior to 3 months. Patients with biliary atresia really only have atresia of the ducts outside the liver (absence of extrahepatic ducts), in fact they have proliferation o f the intrahepatic ducts. They will develop cirrhosis without treatment and not do well

Answer 350

• Associations with Polysplenia, and Trisomy 18 • Gallbladder may be absent (normal gallbladder - supports neonatal hepatitis) • Triangle Cord Sign - triangular echogenic structure by the portal vein - possibly remnant of the CBD. • Hepatobiliary Scintigraphy with 99m Tc-IDA is the test o f choice to distinguish (discussed in the Nukes Chapter). • Alagille Syndrome: This is a total zebra. All you need to know is hereditary cholestasis, from paucity o f intrahepatic bile ducts, and peripheral pulmonary stenosis. The purpose o f a liver biopsy in biliary atresia is to exclude this diagnosis.

Answer 351

If you see a peds patient with gallstones think sickle cell.

Answer 352

These kids bodies have spleen better days - as the spleen will typically enlarge progressively and then eventually auto-infarct and shrink (during the first decade). If the spleen remains enlarged it can run into problems - mainly acute splenic sequestration crisis.

Answer 353

This is the second most common cause o f death in SC patients younger than 10. We are talking about the situation in which the spleen becomes a greedy little pig and tries to hog all the blood for itself.

Answer 354

History o f abdominal pain or vital signs suggesting low volume (high HR, low BP) with a big spleen. Remember most kids with sickle cell will have smaller spleens (auto infarct) so a big spleen should be your clue.

Answer 355

Other problems you can run into if your spleen stays big are abscess formation and large infarcts. These large infarcts are not the same pathophysiology as the “auto-infarct” you typically think of with sickle cell. These are the big wedge shaped infarcts (hypo-perfusion on CT). As a point of trivia, infarcted splenic tissue should look hypoechoic on US, with linear “bright bands ” — google that if you haven’t seen it before.

Answer 356

This is different than the massive infarct in that it is typically the combined effort of numerous tiny, unnoticeable, and repetitive micro occlusions leading to progressive atrophy. Supposedly this doesn’t hurt (large infarcts do). This tends to occur early and is usually “complete" by age 8. The typically look is going to be a tiny (possibly calcified) spleen. When I say tiny - we are talking like 1cm. In the imaginary world of multiple choice you might not even see the fucking thing

Answer 357

Auto Infarct = Sickle Cell

Answer 358

If you don't see the spleen but you do see a gallbladder full o f stones in a kid less than 15 - you should think Sickle Cell.

Answer 359

Heterotaxia syndromes are clutch for multiple choice | tests. The major game played on written tests is “left side vs right side. ”

Answer 360

Let me break this down like a cardboard box that you intend to put in your recycling ... because this gets pretty fucking complicated. I like to start in the lungs. The right side has two fissures (major and minor). The left side has just one fissure. So if 1 show you a CXR with two fissures on each side, (a left sided minor fissure), then the patient has two right sides. Thus the term “bilateral right sidedness.”

Answer 361

The liver. So, these patients won’t have a spleen (the spleen is a left sided structure).

Answer 362

Since the spleen is on the left, a “bilateral left sided” patient will have polysplenia.

Answer 363

This relationship is a little more confusing when you try to reason it out. The way I keep it straight is by remembering that the IVC is usually on the right. If you are “bilateral left” then you don’t have a regular IVC — hence the azygos continuation. Then just remember that the other one (flipped IVC/Aorta) is the other one.

Answer 364

Reversed (Aorta/ ICV) | -Asplenia

Answer 365

Azygos Continuation | -Polysplenia

Answer 366

two fissures in left lung asplenia increased cardiac malformations reversed aorta/ivc

Answer 367

one fissure in right lung polysplenia less cardiac malformations azygos continuation of the ivc

Answer 368

• Situs Solitus - Instead o f just saying normal, you can be an asshole and say “Situs Solitus. ” • Situs Inversus Totalis - Total mirror image transposition o f the abdominal and thoracic stuff. • Situs Ambiguus (Ambiguous) - This is a tricky way of saying Heterotaxy, o f which you can have left or right “isomerism.”

Answer 369

1 guess some asshole really liked organic chemistry.... This is a fancy way of saying bilateral right or bilateral left

Answer 370

``` • Gastric Bubble on Left • Larger part of Liver on Right • Minor fissure on Right ```

Answer 371

``` • Gastric Bubble on Right • Larger part of Liver on Left • Minor fissure on Left • Inverted Bronchial Pattern • Associated with Primary Ciliary Dyskinesia ```

Answer 372

``` • Absent Minor Fissures • Interrupted I VC • Polysplenia • Biliary Atresia ( 10%) ```

Answer 373

• Congenital liver fibrosis -» cholangiopathy -» neonatal jaundice (after 1 week of life) • US: Bright band of tissue (triangular cord sign) near branching of common bile duct; small or absent gallbladder (fasting ~ 3 hours) • Scintigraphic: No tracer excretion into bowel by 24 hours • Biopsy to exclude = Zebra Alagille syndrome • Treated with Kasai procedure

Answer 374

``` • Duodenum to the right of the midline • Increased risk for mid gut volvulus, and internal hernia • “Bilious vomiting” • SMA to the right of the SMV ```

Answer 375

Right Sided: two fissures in left lung asplenia increased cardiac malformations reversed aorta/ivc LEft sided: one fissure in right lung polysplenia less cardiac malformations azygos continuation of the ivc

Answer 376

``` V - Vertebral Anomalies (37%) A - Anal (imperforate anus) (63%) C - Cardiac (77%) TE - Tracheoesophageal Fistula or Esophageal Atresia ( 40%) R - Renal (72%) L - Limb (radial ray) - 58% ```

Answer 377

Show unilateral agenesis on prenatal US - as an absent renal artery (in view of the aorta) or oligohydramnios - with followup questions on associations. Or make it super obvious with a CT / MRI and ask association questions.

Answer 378

• 70% of women with unilateral renal agenesis have associated genital anomalies (usually unicornuate uterus, or a rudimentary horn). • 20% of men are missing the epididymis, and vas deferens on the same side they are missing the kidney. PLUS they have a seminal vesicle cyst on that side.

Answer 379

Insult (maybe ACE inhibitors) = kidneys don’t form, if kidneys don’t form you can’t make piss, if you can’t make piss you can’t develop lungs (pulmonary hypoplasia).

Answer 380

describes the elongated appearance of the adrenal not normally molded by the adjacent kidney. It can be used to differentiate surgical absent vs congenitally absent.

Answer 381

This is the most common fusion anomaly. The kidney gets hung up on the IMA. Questions are most likely to revolve around the complications / risks: • Complications from Position - Easy to get smashed against vertebral body - kid shouldn’t play football or wrestle. • Complications from Drainage Problems: Stones, Infection, and Increased risk of Cancer (from chronic inflammation) - big ones are Wilms, TCC, and the Zebra Renal Carcinoid. • Association Syndrome Trivia - Turner’s Syndrome is the classic testable association.

Answer 382

One kidney comes across the midline and fuses with the other. Each kidney has its own orthotopic ureteral orifice to drain through. It’s critically important to the patient to know that “the Ectopic Kidney is Inferior. ” The left kidney more commonly crosses over to the right fusing to the normal right kidney lower pole. Complications include stones, infection, and hyponephrosis (50%). The classic way to show this is two axial CTs. The first at the level of the kidneys hinting that one kidney may be absent. The second through the bladder (on a delayed phase) showing two opacified ureters.

Answer 383

This is the most common congenital anomaly of the GU tract in neonates. About 20% of the time, these are bilateral. Most (80%) of these are thought to be caused by intrinsic defects in the circular muscle bundle of the renal pelvis. Treatment is a pycloplasty. A Radiologist can actually add value

Answer 384

Whitaker Test ”, which is a urodynamics study combined with an antegrade pyelogram.

Answer 385

Teenager with flank pain after drinking “lots of fluids.”

Answer 386

These do NOT have dilated ureters (NO HYDROURETER).

Answer 387

These guys get HTN and renal failure. The liver involvement is different than the adult form (ADPKD). Instead of cysts they have abnormal bile ducts and fibrosis. This congenital hepatic fibrosis is ALWAYS present in ARPKD. The ratio of liver and kidney disease is inverse. The worse the liver is the better the kidneys do. The better the liver is the worse the kidneys arc. Death is often from portal hypertension.

Answer 388

On ultrasound the kidneys are smoothly enlarged and diffusely echogcnic, with a loss of corticomedullary differentiation. In utero you sometimes will not see urine in the bladder. Cysts tend to be tubular and spare the cortex

Answer 389

Big Bright, with Lost | Corticomedullary Differentiation.

Answer 390

This is an associated condition of maternal diabetes, sepsis, and dehydration. It is typically unilateral (usually left). The theory is that it starts peripherally and progresses toward the hilum. When acute, will cause renal enlargement. When chronic, will result in renal atrophy.

Answer 391

This occurs secondary to umbilical artery | catheters. Unlike renal vein thrombosis it does NOT present with renal enlargement but instead severe hypertension.

Answer 392

This is a malformation triad which occurs in males. Classically shown on a babygram with a kid shaped like a pear (big wide belly).

Answer 393

• Deficiency of abdominal musculature • Hydroureteronephrosis • Cryptorchidism (bladder distention interferes with descent o f testes)

Answer 394

This is a “wastebasket” term for an enlarged ureter which is intrinsic to the ureter (NOT the result of a distal obstruction). Causes include (1) distal adynamic segment (analogous to achalasia, or colonic Hirschsprungs), (2) reflux at the UVJ, (3) it just wants to be big (totally idiopathic). The distal adynamic type “obstructing primary megaureter,” can have some hydro, but generally speaking an absence o f dilation o f the collecting system helps distinguish this from an actual obstruction.

Answer 395

This is actually a problem with the development o f the IVC, which grows in a manner that pins the ureter. Most o f the time it’s asymptomatic, but can cause partial obstruction and recurrent UTI. I VP will show a “reverse J” or “fishhook” appearance o f the ureter.

Answer 396

The main thing to know about duplicated systems is the so-called “Weigert- Meyer Rule” where the upper pole inserts inferior and medially. The upper pole is prone to ureterocele formation and obstruction. The lower pole is prone to reflux. Kidneys with duplicated systems tend to be larger than normal kidneys. In girls, a duplicated system can lead to incontinence (ureter may insert below the sphincter - sometimes into the vagina).

Answer 397

* Upper Pole Obstructs * Lower Pole Refluxes ``` Upper Pole (obstructed / dilated) inserts Inferior and Medial ``` Lower Pole inserts Superior and Lateral

Answer 398

A cystic dilation o f the intravesicular ureter, secondary to obstruction at the ureteral orifice. IVP (or US) will show the “cobra head” sign, with contrast surrounded by a lucent rim, protruding from the contrast filled bladder. This is associated with a duplicated system (specifically the upper pole).

Answer 399

The ureter inserts distal to the external sphincter in the vestibule. More common in females and associated with incontinence (not associated with incontinence in men). Ureteroceles are best demonstrated during the early filling phase o f the VCUG.

Answer 400

This is a fold in the posterior urethra that leads to outflow obstruction and eventual renal failure (if it’s not fixed). It is the most common cause of urethral obstruction in male infants.

Answer 401

The fold is a Wolfian Duct tissue remnant

Answer 402

The key finding on VCUG is an abrupt caliber change between the dilated posterior urethra and normal caliber anterior urethra.

Answer 403

The MR1 would have to show hydro in | the kidney and a “key-hole” bladder appearance.

Answer 404

* Hydronephrosis * Bladder Dilation * Oligiohydramnios

Answer 405

“Peri-renal fluid collection” is a buzzword, and it’s the result o f fomiceal rupture. Obviously that is non-specific and can be seen with any obstructive pathology.

Answer 406

- Vesicoureteral Reflux (VUR) - Primary Megaureter - Prune Belly = Zebra

Answer 407

- PUV - UPJ Obstruction - Ureteral Ectopia

Answer 408

Hydronephrosis on Routine Prenatal Screening > repeat ultrasound once born > goes away in most > if persistent > VCUG to ealuate anatomy (gold standard), adjunctive MAG 3 to evaluate function and drainage (obstructive vs non obstructive), MR urography offers functionand structure but requires sedation in little kids

Answer 409

Normally, the ureter enters the bladder at an oblique angle so that a “valve” is developed. If the angle of insertion is abnormal (horizontal) reflux can develop. This can occur in the asymptomatic child, but is seen in 50% o f children with UTIs. The recommendations for when the boy/girl with a UTI should get a VCUG to evaluate for VUR is in flux (not likely to be tested). Most of the time VUR resolves by age 5-6.

Answer 410

Hydronephrosis is the most common | cause of a palpable renal “mass" in childhood.

Answer 411

There is a grading system fo r VUR which goes 1-5. One is reflux halfway up the ureter, Two is reflux into a non-dilated collecting system, (calyces still pointy), Three you have dilation o f the collecting system, and calyces get blunted Four the system gets mildly tortuous, Five the system is very tortuous

Answer 412

A sneaky trick would be to show the echogenic mound near the UVJ, that results from injection o f “deflux”, which is a treatment urologist try. Essentially, they make a bubble with this proprietary compound in the soft tissues near the UVJ and it creates a valve (sorta). Anyway, they show it in a lot o f case books and textbooks so just like a midget using a urinal - remember to stay on your toes.

Answer 413

Chronic reflux can lead to scarring. This scarring can result in hypertension and/or chronic renal failure

Answer 414

If the reflux appears to be associated with a “hutch” diverticulum - people will use the vocabulary “Secondary” VUR rather than Primary VUR. The treatment in this case will be surgical. Ureteroceles, Posterior Valves, Neurogenic Bladder - are all causes of Secondary VUR.

Answer 415

-Intravesicular | ureter is oblique

Answer 416

intraversicular ureter is horizontal

Answer 417

* Occur at or adjacent (usually just above) the UVJ. * Caused by congenital muscular defect * Difficult to see on US — better seen with VCUG. * They are “dynamic” and best seen on the voiding (micturition) * If associated with VUR will often be surgically resected

Answer 418

The umbilical attachment to the bladder (started out being called the allantois, then called the Urachus). This usually atrophies into the umbilical ligament. Persistent canalization can occur along a spectrum (patent, sinus, diverticulum, cyst).

Answer 419

Most common complication of | urachal remnant = infection

Answer 420

Urachal anomalies are twice as common in boys | relative to girls

Answer 421

The most important piece of trivia is that when these guys get cancer, it’s adenocarcinoma (90% of cases). To hint at this multiple choice test writers will often use the phrase “midline bladder structure

Answer 422

Normal Obliterated Urachus (only the ligament remains) Patent Urachus Vesicourachal Diverticulum Urachal Cyst Umbilical Urachal Sinus

Answer 423

This is a herniation of the urinary | bladder through a hole in the anterior infra-umbilical abdominal wall.

Answer 424

• Increased incidence of Malignancy in the extruded bladder • It’s Adenocarcinoma - just like a Urachal Remnant would get • Aunt Minnie “Manta Ray Sign” - with unfused pubic bones. This looks like a monster wide pubic symphysis on an AP pelvic radiograph.

Answer 425

gu and gi both drain into a common openeing (like a birk) this only happens in females

Answer 426

1 will discuss this more in the adult Urinary chapter, but for kids I want you to think about spinal dysraphism (tethered cord, sacral agenesis, and all the other fucked up spine stuff).

Answer 427

nephroblastomasosis mesoblastic nephroma

Answer 428

wilms wilms variants lymphoma multilocular cystic nephroma

Answer 429

rcc lymphoma

Answer 430

Solid Tumor o f Infancy ” (you can be born with it)

Answer 431

“Nephrogenic Rests” - left over embryologic crap that didn’t go away Might turn into wilms (bilateral wilms especially) “Next Step” - f/u ultrasound till 7-8 years old Variable appearance

Answer 432

90% + Renal Tumors “Solid Tumor o f Childhood” - Never born with it Grows like a solid ball (will invade rather than incase) Met to the lung (most common)

Answer 433

met to bone

Answer 434

Brain Tumors | It fucks you up, it takes the money (it believes in nothing Lebowski)

Answer 435

Micheal Jackson Tumor (Young Boys, Middle Age Women) Big cysts that don’t communicate Septal Enhancement Can't Tell it is not Cystic Wilms (next step = resection)

Answer 436

“Solid Tumor o f Adolescent” | Syndromes - VHL, TS

Answer 437

Non-Hodgkin | Multifocal

Answer 438

Nephroblastomatosis Mesoblastic Nephroma

Answer 439

These are persistent nephrogenic rests beyond 36 weeks. It’s sorta normal (found in 1% of infants). But, it can be a precursor to Wilms so you follow it. When Wilms is bilateral, 99% of the time it had nephroblastomatosis first. It goes away on its own (normally). It should NOT have necrosis - this makes you think Wilms. It has a variable appearance, and is often described as “homogeneous.” Although more commonly a focal homogeneous ball, the way it’s always shown in case conferences and case books is as a hypodense rind. Ultrasound screening q 3 months till age 7-8 is the usual routine - to make sure it doesn’t go Wilms on you. -Hypodense Rind -

Answer 440

“Solid renal tumor o f infancy." This is a fetal hamartoma, and generally benign. It is the most common neonatal renal tumor (80% diagnosed in the first month on life). Often involves the renal sinus. Antenatal ultrasound may have shown polyhydramnios. Pearl: If it really looks like a Wilms, but they are just too young (< 1 year) then call it mesoblastic nephroma.

Answer 441

Multicystic Dysplastic Kidney

Answer 442

You have multiple tiny cysts forming in utereo. What you need to know is (1) that there is “no functioning renal tissue,” (2) contralateral renal tract abnormalities occur like 50% o f the time (most commonly UPJ obstruction).

Answer 443

* In hydronephrosis, the cystic spaces are seen to communicate. * In difficult cases renal scintigraphy can be useful. MCDK will show no excretory function.

Answer 444

MCDK has MACROscopic cysts that do NOT communicate

Answer 445

• Beckwith-Wiedemann - Macroglossia (most common finding), Omphalocele, Hemihypertrophy, Cardiac, Big Organs. • Sotos - Macrocephaly, Retarded (CNS stuff), Ugly Face

Answer 446

• WAGR - Wilms, Aniridia, Genital, Growth Retardation • Drash - Wilms, Pseudohermaphroditism, Progressive Glomerulonephritis

Answer 447

About 5-10% will have bilateral disease ( “Synchronous Bilateral Wilms ”)

Answer 448

(look ju s t like Wilms) • Clear Cell - likes to go to bones (lytic) • Rhabdoid - “Terrible Prognosis” - Associated with aggressive Rhabdoid brain tumors

Answer 449

• NEVER Biopsy suspected Wilms (you can seed the tract and up the stage) • Wilms NEVER occurs before 2 months of age (Neuroblastoma can)

Answer 450

Think about associated syndromes. Wilms loves to pal around with: • Hemihypertrophy, • Hypospadias, • Cryptorchidism

Answer 451

You Say, •Wilms, •Omphalocele, •Hepatoblastoma

Answer 452

Multilocular Cystic Nephroma

Answer 453

“Non-communicating, fluid-tilled locules, surrounded by thick fibrous capsule.” By definition these things are characterized by the absence of a solid component or necrosis. There is a classic bimodal occurrence (4 year old boys, and 40 year old women). I like to think o f this as the Michael Jackson lesion - it loves young boys and middle-aged women

Answer 454

“protrude into the renal pelvis.”

Answer 455

One of my favorite jokes has been this “Michael Jackson lesion” to help remember the age distribution. Unfortunately - a bunch of Academic Nerds got together and decided that the pediatric cystic nephromas arc their own thing now, and the adult ones are their own thing - with the words "adult cystic nephromas” and "pediatric cystic nephroma” as the preferred nomenclature. Assholes.... ruined a great joke.

Answer 456

Board exams usually lag a few years behind these kinds of changes. So if you get a question asking about the age distribution, you may want to still go with the bimodal occurrence - especially if they don’t say “adult" or “peds” — just make sure there isn’t another more correct answer. This is where reading all the choices is critically important. Mind reading is also helpful. Don’t forget to read the mind of the person who wrote the question - so you can understand his or her bias.

Answer 457

Renal Lymphoma and RCC can occur in teenagers. Renal lymphoma can occur in 5 year olds as well.

Answer 458

Renal Lymphoma and RCC can occur in teenagers. Renal lymphoma can occur in 5 year olds as well.

Answer 459

This is the most common bladder cancer in humans less than 10 years of age. They are often infiltrative, and it’s hard to tell where they originate from. “Paratesticular Mass” is often a buzzword. They can met to the lungs, bones, and nodes. The Botryoid variant produces a polypoid mass, which looks like a bunch of grapes. I'll discuss this again in the testicle section.

Answer 460

Isn’t a Renal Mass, but is frequently contrasted with Wilms so I want to discuss it in the renal section. It is the most common extra-cranial solid childhood malignancy. They typically occur in very young kids (you can be born with this). 95% of cases occur before age 10. They occur in the abdomen more than the thorax (adrenal 35%, retroperitoneum 30%, posterior mediastinum 20%, neck 5%).

Answer 461

Things that up the stage include crossing the midline, and contralateral positive nodes. These things make it Stage 3.

Answer 462

Diagnosis in Age < 1, Thoracic Primary, Stage 4S.

Answer 463

* NF-1, Hirschsprungs, DiGeorge, Beckwith Wiedemann | * Most are sporadic

Answer 464

• Opsomyoclonus (dancing eyes, dancing feet) - paraneoplastic syndrome associated with neuroblastoma. • “Raccoon Eyes” is a common way for orbital neuroblastoma mets to present • MIBG is superior to Conventional Bone Scan for Neuroblastoma Bone Mets • Neuroblastoma bone mets are on the “lucent metaphyseal band DDx” • Sclerotic Bone mets are UNCOMMON • Urine Catecholamines are always (95%) elevated

Answer 465

• Less than 1 year old • Distal Mets are Confined to Skin, Liver, and Bone Marrow • Excellent Prognosis. **A common distractor is to say 4S goes to cortical bone. This is false! It’s the marrow.

Answer 466

Age: usually less than 2 (can occur in utero) calcifies 90% encases vessels (doesnt invade) poorly marginated mets to bones

Answer 467

Age: Usually around age 4 (never before 2 months) Calcifies Rarely (<10%) Invades Vessels (doesn’t encase) Well Circumscribed Doesn’t usually met to bones (unless clear cell Wilms variant). Prefers lung.

Answer 468

This can occur in the setting o f birth | trauma or stress.

Answer 469

Neonatal adrenal hemorrhage is associated with scrotal hemorrhage

Answer 470

• Ultrasound can usually tell the difference (adrenal hemorrhage is anechoic and avascular, neuroblastoma is echogenic and hyper-vascular). • MRI could also be done to problem solve if necessary (Adrenal Hemorrhage low T2 , Neuroblastoma high T2).

Answer 471

Neonates that are sick enough to be in the hospital hemorrhage their fucking adrenals all the time. An adrenal hemorrhage can look just like a mass on ultrasound. Yes, technically it should be anechoic and avascular - but maybe your tech sucks, or maybe you don’t get shown a picture they just tell you it’s a mass. The question writer is most likely going to try and trick you into worrying about a neuroblastoma (which is also going to be a mass in the adrenal).

Answer 472

Sticking a needle in it, sedating the kid for MRI, or exposing him/her to the radiation o f CT, PET, or MIBG are all going to go against the “image gently” propaganda being pushed at academic institutions. Plus it’s unnecessary. As is true with most things in radiology, they either get better or they don’t. Hemorrhage is going to resolve. The cancer is not. So the first step is going to be followup ultrasound imaging

Answer 473

Nope. Most cases are actually caused by a 21 alpha hydroxylase deficiency (congenital adrenal hyperplasia). Those tend to look different than hemorrhage or a mass- they are more “cerebriform.” The problem is you can acquire adrenal insufficiency from neoplastic destruction (neuroblastoma) or regular good old fashioned hemorrhage.

Answer 474

Essentially the vagina won’t drain the uterus. This condition is characterized on imaging by an expanded fluid-filled vaginal cavity with associated distention of the uterus.

Answer 475

You can see it presenting in infancy as a mass, or as a | teenager with delayed menarche.

Answer 476

Causes include imperforate hymen (most common), vaginal stenosis, lower vaginal atresia, and cervical stenosis

Answer 477

For multiple choice trivia think about this as a “midline pelvic mass” , which can cause hydronephrosis (mass effect from distended uterus).

Answer 478

Trivia: Associated with Uterus Didelphys (which often | ~75% has a transverse vaginal septum)

Answer 479

In an adult, ovarian torsion is almost always due to a mass. In a child, torsion can occur with a normal ovary, secondary to excessive mobility o f the ovary. As described in the GYN chapter you are going to see an enlarged (swollen) ovary, with peripheral follicles, with or without arterial flow. What is “enlarged” ? Unlike an adult you can’t really use a fixed number to call the ovary enlarged (ovarian volumes in the peds setting are notoriously variable). The solution is to compare the ovary in question to the contralateral size. Suspect torsion if the ovary is at least 3 times the size of the opposite “normal” ovary. Fluid-Debris Levels within the displaced follicles is another described adolescent ovarian torsion finding.

Answer 480

About two-thirds of ovarian neoplasms are benign dermoids/teratomas (discussed in detail in the GYN chapter). The other one third are cancer. The cancers are usually germ cells (75%). Again, mural nodules and thick septations should clue you in that these might be cancer. Peritoneal implants, ascites, and lymphadenopathy, are all bad signs and would over-ride characteristics of the mass.

Answer 481

Collection of serous fluid and is the most common cause of painless scrotal swelling. Congenital hydroceles result from a patent processus vaginalis that permits entry of peritoneal fluid into the scrotal sac.

Answer 482

This is either a hematocele vs pyocele. The distinction is clinical.

Answer 483

Most of these are idiopathic and found in adolescents and young adults. They are more frequent on the left. They are uncommon on the right, and if isolated (not bilateral) should stir suspicion for abdominal pathology (nutcracker syndrome, RCC, retroperitoneal fibrosis).

Answer 484

Isolated right-sided varicocele = Abdomen CT Exclude the extrinsic mass, renal vein thrombus, or portal hypertension causing a splenorenal shunt

Answer 485

This vasculitis is the most common cause of idiopathic scrotal edema

Answer 486

The top three considerations in a child with acute scrotal pain are (1) torsion of the testicular appendage, (2) testicular torsion, and (3) epididymo-orchitis.

Answer 487

The epididymal head is the most common part involved. Increased size and hyperemia are your ultrasound findings. This occurs in two peaks: under 2 and over 6. You can have infection of the epididymis alone or infection of the epididymis and testicle (isolated orchitis is rare).

Answer 488

Nearly always occurs as a progressed epididymitis. When isolated the answer is mumps.

Answer 489

This is the most common cause of acute scrotal pain in age 7-14. The testicular appendage is some vestigial remnant of a mesonephric duct. Typical history is a sudden onset of pain, with a Blue Dot Sign on physical exam (looks like a blue dot). Enlargement of the testicular appendage to greater than 5 mm is considered by some as the best indicator of torsion

Answer 490

Results from the testis and spermatic cord twisting within the serosal space leading to ischemia. The testable trivia is that it is caused by a failure o f the tunica vaginalis and testis to connect or a “Bell Clapper Deformity”. This deformity is usually bilateral, so if you twist one they will often orchiopexy the other one. If it was 1950 you’d call in your nuclear medicine tech for scintigraphy. Now you just get a Doppler ultrasound. Findings will be absent or asymmetrically decreased flow, asymmetric enlargement, and slightly decreased echogenicity of the involved ball.

Answer 491

By far the most common extra-testicular mass in young men and the only one really worth mentioning. If you see a mass in the scrotum that is not for sure in the testicle this is it (unless the history is kick to the balls from a spiteful young lady - and you are dealing with a big fucking hematoma). If it’s truly a mass - this is the answer.

Answer 492

• The most common location is actually the head/neck - specifically the orbit and nasopharynx. • There is a bimodal peak (2-4, then 15-17).

Answer 493

Testicular Masses can be thought of as intratesticular or extratesticular. With regard to intratesticular masses, ultrasound can show you that there is indeed a mass but there are no imaging features that really help you tell which one is which. If the mass is extratesticular , the most likely diagnosis is an embryonal rhabdomyosarcoma from the spermatic cord or epididymis

Answer 494

This appears as multiple small echogenic foci within the testes. Testicular microlithiasis is usually an incidental finding in scrotal US examinations performed for unrelated reasons. It might have a relationship with Germ Cell Tumors (controversial). Follow-up in 6 months, then yearly is probably the recommendation (maybe - it’s very controversial, and therefore unlikely to be asked).

Answer 495

Germ Cell (90%) • Seminoma (40%) - seen more in the 4th decade • Non Seminoma (60%) • Teratoma, Yolk Sacs, Mixed Germ Cells, Etc. Non Germ Cell (10%) •Sertoli •Leydig

Answer 496

Tiny (micro) = Seminoma | BIG = Germ Cell Tumor

Answer 497

yolk sac tumor, and the | teratoma.

Answer 498

Heterogeneous Testicular Mass in < 2 year old = Yolk Sac Tumor. AFP is usually super elevated.

Answer 499

Pure testicular teratomas are only seen in young kids < 2. Mixed teratomas are seen in 25 year olds. Unlike ovarian teratoma, these guys often have aggressive biological behavior.

Answer 500

An aggressive, highly vascular tumor, seen more in the 2nd decade.

Answer 501

These testicular tumors are usually bilateral and are visualized on US as “burned-out” tumors (dense echogenic foci that represent calcified scars). A subtype of Sertoli cell tumor associated with Peutz-Jeghers syndrome typically occurs in children. If they show you the Peurtz-Jegher lips and bilateral scrotal masses, this is the answer.

Answer 502

Just be aware that lymphoma can “hide” in the testes because of the blood testes barrier. Immunosuppressed patients are at increased risk for developing extranodal/ testicular lymphoma. On US, the normal homogeneous echogenic testicular tissue is replaced focally or diffusely with hypocchoic vascular lymphomatous tissue. Buzzword = multiple hypoechoic masses of the testicle.

Answer 503

This is the most common tumor of the fetus or infant. These solid and/or cystic masses are typically large and found either on prenatal imaging or birth. Their largeness is a problem and can cause mass effect on the GI system, hip dislocation, and even nerve compression leading to incontinence. They are usually benign (80%), although those presenting in older infants tend to have a higher malignant potential. The location of the mass is either external to the pelvis (47%), internal to the pelvis (9%), or dumbelled both inside and outside (34%).

Answer 504

The easiest way to remember it is like this: - Type 1 - Totally extra pelvic - Type 2 - Barely pelvic, but not abdominal - Type 3 - Some abdominal - Type 4 - Totally inside abdomen ** this one has the highest rate o f malignancy.

Answer 505

They have to cut the coccyx off during resection. Incomplete resection of the coccyx is associated with a high recurrence rate.

Answer 506

In general, little kids bend they don’t break. You end up with lots of buckles and greensticks. For problem solving you can get a repeat in 7-10 days as periosteal reaction is expected in 7-10 days. Kids tend to heal completely, often with no sign of prior fracture.

Answer 507

The major concern is growth arrest, probably best asked by showing a physeal bar (“early” bony bridge crossing the growth plate). You can get bars from prior infection, but a history of trauma is gonna be the more classic way to ask it.

Answer 508

S - Slipped Complete physeal fracture, with or without displacement.

Answer 509

A - Above (or “Away from the Joint”) Fracture involves the metaphysis. This is the most common type (75%).

Answer 510

L - Lower (3 is the backwards “E ”fo r Epiphysis) Fracture involves the epiphysis. These guys have a chance of growth arrest, and will often require surgery to maintain alignment

Answer 511

T - Through Fracture involves the metaphysis and epiphysis. These guys don’t do as well, often end up with growth arrest, or focal fusion. They require anatomic reduction and often surgery.

Answer 512

R - Ruined Compression of the growth plate. It occurs from axial loading injuries, and has a very poor prognosis. These are easy to miss, and often found when looking back at comparisons (hopefully ones your partner read). The buzzword is “bony bridge across physis”.

Answer 513

Oblique fracture o f the midshaft o f the tibia seen in a child just starting to walk (new stress on bone). If it’s a spiral type you probably should query nonaccidental trauma. The typical age is 9 months - 3 years.

Answer 514

This is an injury which occurs after repetitive trauma, usually after new activity (walking). The most common site o f fracture is the tibia - proximal posterior cortex. The tibial fracture is the so-called “toddler fracture” described above. Other classic stress fractures include the calcaneal fracture - seen after the child has had a cast removed and returns to normal activity.

Answer 515

Every first year resident knows that elevation o f the fat pad (sail sign) should make you think joint effusion and possible occult fracture. Don’t forget that sometimes you can see a thin anterior pad, but you should never see the posterior pad (posterior is positive). I like to bias myself with statistics when I’m hunting for the peds elbow fracture. The most common fracture is going to be a supracondylar fracture (>60%), followed by lateral condyle (20%), and medial epicondyle (10%).

Answer 516

``` This is a line through the center of the radius, which should intersect the middle of the capitellum on every view (regardless of position). If the radius is dislocated it will NOT pass through the center of the capitellum ```

Answer 517

``` This time you need a true lateral. A line along the anterior surface of humerus, should pass through the middle third of the capitellum. With a supracondylar fracture (the most common peds elbow fracture) you’ll see this line pass through the anterior third. ```

Answer 518

``` Rr . emember they occur in a set order (c r it o e ) • Capitellum (Age 1) • Radius (Age 3), • Internal (medial epicondyle Age 5), • Trochlea (Age 7), • olecranon(Age 9), • External (lateral epicondyle Age 11). ```

Answer 519

This is the second most common distal humerus fracture in kids. Some dude named Milch classified them. The thing to know is a fracture that passes through the capitello-trochlear groove is unstable (Milch II). Since it’s really hard to tell this, treatment is based on the displacement of the fracture fragment (> or < 2mm).

Answer 520

can have multiple ossification centers, so it can have a fragmented appearance

Answer 521

h e re are two major tricks with this one. (1) Because it’s an extra-articular structure, its avulsions will not necessarily result in a joint effusion. (2) It can get interposed between the articular surface of the humerus and olecranon. Avulsed fragments can get stuck in the joint, even when there is no dislocation.

Answer 522

When a child’s arm is pulled on, the radial head may sublux into the annular ligament. X-rays typically don’t help, unless you supinate the arm during lateral position (which often relocates the arm).

Answer 523

* Is the patient 5 years old ? And if so | * Where is the medial epicondyle ?

Answer 524

• You should never see the trochlea and not see the internal (medial epicondyle), if you do it’s probably a displaced fragment

Answer 525

lateral condylar medial epicondyle

Answer 526

lateral epicondyle | medial epicondyle

Answer 527

abdominal muscles

Answer 528

rectus femoris

Answer 529

gluteal muscles

Answer 530

hamstrings

Answer 531

Sinding-Larsen-Johansson Osgood-Schlatter

Answer 532

This is a chronic traction injury at the insertion of the patellar tendon on the patella. It’s seen in active adolescents between age 10-14. Kids with cerebral palsy are prone to it.

Answer 533

This is due to repeated micro trauma to the patellar tendon on its insertion at the tibial tuberosity. It’s bilateral 25% of the time, and more common in boys

Answer 534

``` Congenital Rubella Syphilis Caffey Disease Prostaglandin Therapy Neuroblastoma Mets Physiologic Growth Abuse ```

Answer 535

Bony changes are seen in 50% of cases, with the classic buzzword being “celery stalk” appearance, from generalized lucency of the metaphysis. This is usually seen in the first few weeks o f life

Answer 536

Bony changes are seen in 95% of cases. Bony changes do NOT occur until 6-8 weeks o f life (Rubella changes are earlier). Metaphyseal lucent bands and periosteal reaction along long bones can be seen. The classic buzzword is “Wimberger Sign” or destruction of the medial portion of the proximal metaphysis of the tibia.

Answer 537

Have you ever seen that giant multiple volume set o f peds radiology books? Yeah, same guy. This thing is a self limiting disorder of soft tissue swelling, periosteal reaction, and irritability seen within the first 6 months of life. The classic picture is the really hot mandible on bone scan. The mandible is the most common location (clavicle, and ulna are the other classic sites). It’s rare as hell, and probably not even real. There have been more sightings o f Chupacabra in the last 50 years.

Answer 538

Prostaglandin El and E2 (often used to keep a PDA open) can cause a periosteal reaction. The classic trick is to show a chest x-ray with sternotomy wires (or other hints of congenital heart), and then periosteal reaction in the arm bones.

Answer 539

This is really the only childhood malignancy that occurs in | newborns and mets to bones.

Answer 540

So this is often called “Physiologic Periostitis of the Newborn” , which is totally false and wrong. It does NOT happen in newborns. You see this around 3 months of age, and it should resolve by six months. Proximal involvement (femur) comes before distal involvement (tibia). It always involves the diaphysis.

Answer 541

Some people abuse drugs, some just can't stand screaming kids, some suffer both shortcomings. More on this later.

Answer 542

* You see it before I month * You see it in the tibia before the femur * It does not involve the diaphysis.

Answer 543

Also known as EG (eosinophilic | granuloma). It’s twice as common in boys. Skeletal manifestations are highly variable

Answer 544

Most common site. Has “beveled edge” from uneven destruction of the inner and outer tables. If you see a round lucent lesion in the skull o f a child think this (and neuroblastoma mets).

Answer 545

Multiple lucent lesions, with an expanded appearance

Answer 546

Vertebra plana

Answer 547

It usually occurs in babies (30% o f cases less than 2 years old). It’s usually hematogenous (adults it directly spreads - typically from a diabetic ulcer). There are some changes that occur over time, which are potentially testable.

Answer 548

They have open growth plates and perforating vessels which travel from the metaphysis to the epiphysis. Infection typically starts in the metaphysis (it has the most blood supply because it is growing the fastest), and then can spread via these perforators to the epiphysis.

Answer 549

Later in childhood, the perforators regress and the avascular epiphyseal plate stops infection from crossing over. This creates a “septic tank” scenario, where infection tends to smolder. In fact, 75% of cases involve the metaphyses o f long bones (femur most common).

Answer 550

When the growth plates fuse, the barrier of an avascular plate is no longer present, and infection can again cross over to the epiphysis to cause mayhem.

Answer 551

- Hematogenous spread more common in kids (direct spread in adult) - Metaphysis most common location, with target changes as explained above - Bony changes don’t occur on x-ray for around 10 days. - It’s serious business and can rapidly destroy the cartilage if it spreads into the joint

Answer 552

Brachydactyly

Answer 553

Polydactyly

Answer 554

Syndactyly

Answer 555

Camptodactyly

Answer 556

Clinodactyly

Answer 557

Arachnodactyly

Answer 558

Rhizomelic

Answer 559

Micromelic

Answer 560

This is the most common skeletal dysplasia, and is the mostly likely to be seen at the mall (or on television). It results from a fibroblast growth factor receptor problem (most dwarfisms do). It is a rhizomelic (short femur, short humerus) dwarf. They often have weird big heads, trident hands (3rd and 4th fingers are long), narrowing o f the interpedicular distance, and the tombstone pelvis. Advanced paternal age is a risk factor. They make good actors, excellent rodeo clowns, and various parts o f their bodies (if cooked properly) have magical powers.

Answer 561

This is the most common lethal dwarfism. They have rhizomelic shortening (humerus, femur). The femurs are sometimes called telephone receivers. They have short ribs and a long thorax, and small iliac bones. The vertebral bones are flat (platyspondyly), and the skull can be cloverleaf shaped

Answer 562

This is usually fatal as well. The big finding is the “Bell shaped thorax” with short ribs. 15% will have too many fingers (polydactyly). If they live, they have kidney problems (chronic nephritis). You can differentiate a dead Thanatophoric dwarf, from a dead Jeune dwarf by looking at their vertebral bodies. The Jeune bodies are normal (the thanatophorics are flat).

Answer 563

is the dwarf with multiple fingers.

Answer 564

is this weird thing not present at birth, and spares the skull.

Answer 565

osteopetrosis, in a dwarf with a wide angled jaw, & Acro-osteolysis

Answer 566

1. The Vocab: Rhizo (humerus, femur) vs Aero (hands, feet) vs Meso (forearm, tib/fib) 2. Most dwarfs are Rhizomelic - if forced to choose, always guess this 3. The pedicles are supposed to widen slightly as you descend the spinal column, Achondroplasia has the opposite - they narrow. If you see a live dwarf, with short femurs / humerus, and narrowing of the pedicles then this is the answer. (technically thantophorics can get this too - but it s more classic fo r achondroplasia) 4. Thanatophoric is your main dead dwarf. Usually the standout feature is the telephone receiver femur (and a crazy cloverleaf head) 5. Jeune is another dead dwarf - but the short ribs really stand out.

Answer 567

This is the most common cause of an anterior wall “mass.” If there is just one (usually the 4th rib) - then it is just a variant. If there are bunches think Gorlin Syndrome.

Answer 568

Bifid Ribs, Calcifications of the Falx, basal cell cancers, odontogeni keratocysts (lytic jaw lesions).

Answer 569

They have a collagen defect and make brittle bones. Depending on the severity it can be totally lethal or more mild. It’s classically shown with a totally lucent skull, 01 multiple fractures with hyperplastic callus. Another classic trick is to show the legs with the fibula longer than the tibia. They have wormian bones, and often flat or beaked vertebral bodies. Other trivia is the blue sclera, hearing impairment (otosclerosis), and that they tend to suck at football.

Answer 570

They have a defect in the way osteoclasts work, so you end up with disorganized bone that is sclerotic and weak (prone to fracture). There are a bunch of different types, with variable severity. The infantile type is lethal because it takes out your bone marrow. With less severe forms, you can have abnormal diminished osteoclastic activity that varies during skeletal growth, and results in alternating bands of sclerosis parallel to the growth plate. Most likely the way this will be shown is the “bone-in-bone” appearance in the vertebral body or carpals. Picture frame vertebrae is another buzzword. Alternatively, they can show you a diffusely sclerotic skeleton, with diffuse loss of the corticomedullary junction in the long tubular bones.

Answer 571

Osteopetrosis + Wormian Bones + Acro-Osteolysis. They also | have "wide (or obtuse) angled mandible ”, which apparently is a buzzword.

Answer 572

You get congenital fusion of the cervical spine (sorta like JRA). The cervical vertebral bodies will be tall and skinny. There is often a sprengel deformity (high riding scapula). Another common piece of trivia is to show the omovertebral bone - which is just some big stupid looking vertebral body.

Answer 573

All three of these are mucopolysaccharidoses. Findings include oval shaped vertebral bodies with anterior beak. The beak is actually mid in Morquio, and inferior in Hurlers. Clavicles and ribs are often thick (narrow more medially) - like a canoepaddle. The pelvis shape is described as the opposite of achondroplasia - the iliac wings are tall and Haired. The hand x-ray is the most commonly shown in case books and gives you wide metacarpal bones with proximal tapering.

Answer 574

* They are dwarfs * The most common cause of death is cervical myelopathy at C2 * The bony changes actually progress during the first few years of life

Answer 575

Just briefly remember that type 1 can cause anterior tibial bowing, and pseudarthrosis at the distal tibula. This is an Aunt Minnie. They often have scoliosis. Just think of the elephant man

Answer 576

This is the most common lysosomal storage disease. It gives you a big spleen, and big liver among a few bone signs. *A VN o f the Femoral Fleads • FI-Shaped Vertebra • Bone Infarcts (lots o f them) •Erlenmeyer Flask Shaped Femurs

Answer 577

This is a spectrum that includes sacral and/or coccyx agenesis. You see it with VACTERL and Currarino Triads Syndromes.

Answer 578

Lateral curvature of the spine, which is usually idiopathic in girls. It can also be from vertebral segmentation problems. NF can cause it as well (that’s a piece of trivia).

Answer 579

``` Absence or hypoplasia of the radius (usually with a missing thumb) is a differential case (VACTERL, Holt-Oram, Fanconi Anemia, Throbocytopenia Absent Radius). As a point of trivia TAR kids will have a thumb. ```

Answer 580

The classic history is hand or foot pain / swelling in an infant with sickle cell. This is a dactylitis, and felt to be related to ischemia. It will resolve on its own, after a few weeks. Radiographs can show a periostitis two weeks after the pain goes away.

Answer 581

tibia vara). Varus angulation occurring at the medial aspect of the proximal tibia (varus bowing occurs at the metaphysis not the knee). This is often bilateral, and NOT often seen before age 2 (two sides, not before two). Later in the disease progression the medial metaphysis will be depressed and an osseous outgrowth classically develops. You can see it in two different age groups; (a) early - which is around age 2 and (b) late - which is around age 12. Two Sides - Not Before Two Two Different Ages (2-3, 12)

Answer 582

Foot or Acquired

Answer 583

Plantar Flexed Ankle ”, Heel Cord is often tight, and the heel won't touch the floor

Answer 584

Opposite o f Equines. The Calcaneus is actually angled up

Answer 585

Forefoot in

Answer 586

Forefoot out

Answer 587

Opposite o f Cavus - “bizarro cavus” - FLAT FOOT

Answer 588

Inward rotation - "Sole offoot in ” - holding soup with the bottom o f your foot

Answer 589

Outward rotation - “Sole offoot out”

Answer 590

``` Think about this as the talus sliding nose down off the calcaneus. This make the angle wider. If the talus slides off you lose your longitudinal arch - which essentially characterizes hindfoot valgus. Also, note that the nose down (nearly vertical) appearance of the talus . “Too Many Toes” ```

Answer 591

hindfoot varus: <25° normal: 25-40° hindfoot valgus: >40°

Answer 592

``` This is the opposite situation, in which you have a narrowing of the angle between the talus and calcaneus. Notice the two bones lay nearly parallel - like two “clubs” laying on top of each other. ```

Answer 593

This can be congenital or acquired. The peds section will cover congenital and the adult MSK section will cover acquired. The congenital types can be grouped into flexible or rigid (the flexible types are more common in kids). The distinction can be made with plantar flexion views (flexible improves with stress). The ridged subtypes can be further subdivided into tarsal coalition and vertical talus. In any case you have a hindfoot valgus

Answer 594

There are two main types (talus to the calcaneus, and calcaneus to the navicular). They are pretty equal in incidence, and about 50% o f the time are bilateral. You can have bony or fibrous/cartilaginous subtypes. The fibrous/cartilaginous types are more common than the bony types.

Answer 595

Occurs at the middle facet. Has the “continuous C-sign ” produced from an “absent middle fac et” on the lateral view. Talar beak (spur on the anterior talus - white arrow) is also seen in about 25% of cases.

Answer 596

Occurs at the anterior facet. Has the “anteater sign ” Where the elongated anterior process o f the calcaneus resembles the blood thirsty nose of a ravenous ant eater. This is best seen on an oblique view.

Answer 597

(equinus hindfoot valgus) - This is sometimes called the “rocker-bottom foot” because the talus is in extreme plantar flexion with dorsal dislocation of the Navicular - resulting in a locked talus in plantar flexion. As a point of trivia this is often associated with myelomeningocele

Answer 598

Translation - Congenital Plantar Flexed Ankle Forefoot. This is sorta why I lead with the vocab, all the congenital feet can be figured out based on the translated language. This thing is more common in boys, and bilateral about half the time. The toes are pointed down (equines), and the talocalcaneal angle is acute (varus).

Answer 599

``` * Hindfoot varus (decreased talocalcaneal angle) * Medial deviation and inversion of the forefoot * Elevated Plantar Arch ```

Answer 600

The most common surgical complication is over correction resulting in a “rocker bottom” flat foot deformity.

Answer 601

This is seen more commonly in females, children bom breech, and oligohydramnios. The physical exam buzzwords are asymmetric skin or gluteal folds, leg length discrepancy, palpable clunk, or delayed ambulation. It’s bilateral about 1/3 of the time. Ultrasound is done to evaluate (after physical exam), and is excellent until the bones ossify (then you need x-rays). A common trick is to be careful making a measurement in the first week o f life - the laxity immediately after birth (related to maternal estrogen) can screw up the measurements.

Answer 602

On ultrasound the alpha angle, should be more than 60 degrees. Anything less than that and your cup is not deep enough to hold your ball. The plain film equivalent in the acetabular angle, which is the complimentary angle (and therefore should be less than 30).

Answer 603

``` Remember that the “Alpha Angle is the Alpha Male’’ - and therefore the bigger of the two angles. **But don’t forget that DDH is more common in women (not alpha males). ```

Answer 604

``` The acetabular angle should decrease from 30 degrees at birth to 22 degrees at age 1. DDH is the classic cause o f an increased angle, but neuromuscular disorder can also increase it. ```

Answer 605

``` The position of the femoral epiphysis (or where it will be) should be below Hilgenreiner’s line “H”, and medial to Perkin’s Line “P”. Shenton’s Line “S” should be continuous. ```

Answer 606

This is a congenital zebra, which ranges from absent proximal femur to hypoplastic proximal femur. You get a varus deformity. This is a mimic o f DDH, but DDH will have normal femur leg length.

Answer 607

This is a type 1 Salter Harris, through the proximal femoral physis. What makes this unique is that unlike most SH Is, this guy has a bad prognosis if not fixed. The classic history is a fat African American adolescent (age 12-15) with hip pain. It’s bilateral in 1/3 o f cases (both hips don’t usually present at same time). The frog leg view is the money - this is always the answer on next step questions.

Answer 608

This is AVN of the proximal femoral epiphysis. It’s seen more in boys than girls (4 :1 ), and favors white people around age 5-8. These kids tend to be smaller than average for their age. This is bilateral about 10% of the time (less than SCFE). The subchondral lucency (crescent sign) is best seen on a frog leg. Other early signs include an asymmetric small ossified femoral epiphysis. MRI has more sensitivity. The flat collapsed femoral head makes it obvious. Sterile joint effusions (transient synovitis) can be associated

Answer 609

Drawn along the edge of the femur and should normally intersect with lateral superior femoral epiphysis. This line is used to evaluate for SCFE. When the line doesn’t cross the lateral epiphysis think SCFE. ***Testable Trivia - Frog Leg View is more sensitive for this measurement

Answer 610

often small white kids ages 5-8 bilateral 10%

Answer 611

overweight black kids age 12-15 bilateral 30%

Answer 612

This is serious business , and considered the most urgent cause o f painful hip in a child. Wide joint space (lateral displacement of femoral head), should prompt an ultrasound, and that should prompt a joint tap. If you have low suspicion and don’t want to tap the hip, You could pull on the leg under fluoro and try and get gas in the joint. This air arthrogram sign supposedly excludes a joint effusion (and therefore a septic joint) - depending on who you ask.

Answer 613

This is a sterile (reactive) hip effusion that occurs in the setting of a systemic illness (usually viral UR1 or GI). As the name suggests this is “transient” and goes away in a few days. This is actually very common. Some sources will say its the most common hip disorder in growing children (peak age is around 5). The ED will be in a full panic and want you to tap it at 3 am.

Answer 614

Telling these apart is actually important for real life (not getting sued) since a septic hip will fucking destroy the kid’s cartilage (usually if it’s missed for more than 4 days). Ortho (and in very rare situations a “smart” ED doc) will use a clinical parameter “the Kocher Criteria ” to tell them apart.

Answer 615

• Fever • Inability to walk • Elevated ESR (or CRP) . WBC > 12K

Answer 616

``` • If 3/4 are positive = Septic • If CRP is negative and the kid can bear weight it’s NOT Septic • CRP is the strongest independent risk factor for septic arthritis ```

Answer 617

1. X-Ray Hip series (AP, Lateral, and Frog Leg) which is usually negative in real life but will probably show medial joint widening on the exam. 2. Ultrasound which will show an effusion. 3. Then a clinical decision based on Kocher Criteria (>2) and “Gut Instinct” to Aspirate 4. MRI would only be used if/when hip aspiration can’t/hasn’t been performed

Answer 618

Not enough vitamin D. Affects the most rapidly growing bones (mostly knees and wrists). Buzzwords “fraying, cupping, and irregularity along the physeal margin. ” They are at increased risk for SCFE. “Rachitic rosary” appearance from expansion of the anterior rib ends at the costochondral junctions. As a pearl, rickets is never seen in a newborn (Mom’s vitamin D is still doing its thing).

Answer 619

This looks like Rickets in a newborn. They will have frayed metaphyses and bowed long bones. The underlying pathology is deficient serum alkaline phosphatase. There is variability in severity with lethal perinatal / natal forms, and more mild adult forms.

Answer 620

Not enough vitamin C. This is rare as hell outside o f a pirate ship in the 1400s. For the purpose of trivia (which multiple choice tests love) the following stuff is high yield: * Does NOT occur before 6 months o f age (maternal stores buffer) o Bleeding Disorders Common o Subperiosteal hemorrhage (lifts up the periosteum) * Hemarthrosis * “Scorbutic rosary” appearance from expansion of the costochondral junctions (very similar to rickets).

Answer 621

This is most commonly seen in kids less than two who eat paint chips. The classic finding is a wide sclerotic metaphyseal line (lead line), in an area o f rapid growth (knee). It will not spare the fibula (as a normal variant line might).

Answer 622

- This is a classic peds DDx. - LINE. * Leukemia * Infection (TORCH) * Neuroblastoma Mets * Endocrine (rickets, Scurvy)

Answer 623

Posterior Medial Rib Fracture Metaphyseal Corner Fractures Skull Fracture Solid Organ and Lumen Injury

Answer 624

In a child under the age o f 3, this is pretty reliable. Supposedly this type o f fracture can only be made from squeezing a child.

Answer 625

When this is present in a non-ambulatory patient (infant) it is HIGHLY specific. The only exception is obstetric trauma. After age 1, this becomes less specific.

Answer 626

The general idea is anything other than a parietal bone fracture (which is supposedly seen more with an actual accident) is concerning.

Answer 627

Don’t forget about this as a presentation for NAT. Duodenal hematoma and pancreatitis (from trauma) in an infant - should get you to say NAT. Just think “belly trauma in a kid that is too young to fall on the handle bars o f their bike

Answer 628

• Periosteal Reaction: This means the fracture is less than a week old. • Complete healing: This occurs in around 12 weeks. • Exceptions: Metaphyseal, skull, and costochondral junction fractures will often heal without any periosteal reaction.

Answer 629

Rickets and 0 1 , can have multiple fractures at different sites and are the two most commonly described mimics. Wormian bones and bone mineral density issues are clues that you are dealing with a mimic. They will have to show you one or the other (or both) if they are gonna get sneaky.

Answer 630

Easily identified as an echogenic line. Although that is sort o f counter intuitive.

Answer 631

The reason is that you are actually seeing a “central echo complex” - which is the interface between the anterior median fissure and the myelinated ventral white matter commissure. Remember that interfaces between things with large differences in impedance cause a lot of reflections (thus an echogenic line).

Answer 632

Technically in a newborn the central canal is not even fluid filled (it’s packed with glial fibrils), but that level of trivia is beyond the scope o f the exam (probably).

Answer 633

Because the canal grows faster than the cord, a fixed attachment (“tethering”) results in cord stretching and subsequent ischemia. This can be primary (isolated), or secondary (associated with myelomeningocele, filum terminale lipoma, or trauma). The secondary types are more likely shown on MR (to showcase the associated mass - fluid collection), the primary types are more likely shown on US - as a straight counting game.

Answer 634

Low conus (below L2L and thickened filum terminale (> 2mm).

Answer 635

A common piece o f trivia used as a distractor is that meningomyelocele is associated with Chiari malformations, lipomyelomeningocele is NOT. * Anal Atresia = High Risk For Occult Cord Problems (including tethering) - should get screened * Low lying / tethered cords are closely linked with Spina Bifida (tufts o f hair) * Low Dimples (below the gluteal crease) Do NOT need screening, o These never extend intra-spinally. They might later become a pilonidal sinuses - but aren’t ever gonna have shit to do with the cord. * High Dimples (above the gluteal crease) DO need screening.

Answer 636

``` Low dimples (below the butt crease) don’t get screened, basically everything else does. Most Likely Question Style: “Which of the following does NOT get screened ?” (Answer = low dimple). ```

Answer 637

This is a developmental variant. Normally, a large portion o f the distal cord involutes in a late stage of spinal cord embryology. Sometimes this process is not uniform and you get stuck with a stupid looking cyst at the end o f your cord. These things are usually small (around 4 mm), and cause no symptoms. Sometimes they can get very big (like this example) and cause some neurologic symptoms.

Answer 638

This is considered a fatigue or stress fracture, probably developing in childhood. It is a classic cause o f back pain in an adolescent athlete. Although they are usually not symptomatic (only 25% are). The process represents a hole / break in the connecting bone between the superior and inferior articular facets. If there is forward “slippage” you can deploy the word spondylolisthesis. Almost always (90% +) you see this at L5 (2nd most common at L4). They tend to have more spondylolisthesis and associated degenerative change at L4-L5 than L5-S1. They can be seen on the oblique plain film as a “collar on the scottie dog.” The collar on the “scotty dog” appearance on an oblique plain film is probably the most common way they show this in case books and conferences. On the AP view this can be a cause o f a sclerotic pedicle (the contralateral pedicle - from wiggle stress). On CT it is usually more obvious with the break clearly demonstrated. **Pars Defects with anterolisthesis will have neuroforaminal stenosis, with spinal canal widening (when severe will have spinal canal stenosis as well). If the process is purely a degenerative spondylolisthesis (not much slippage), the resulting facet arthropathy will favor the canal with less severe effects on the neuro foramina

Answer 639

You can group these as open or closed (closed with and without a mass). Open means neural tissue exposed through a defect in bone and skin (spina bifida aperta). Closed means the defect is covered by skin (spina bifida occulta).

Answer 640

This is the result o f a failure o f the closure of the primary neural tube, with obvious exposure o f the neural placode through a midline defect o f the skin. You have a dorsal defect in the posterior elements. The cord is going to be tethered. There is an association with diastematomyelia and Chiari II malformations. Early surgery is the treatment / standard of care.

Answer 641

This is the more rare type where the neural | placode is flush with the skin.

Answer 642

This is the more common type (98%) where the neural placode protrudes above the skin. These are more common with Chiari II malformations.

Answer 643

This is herniation o f a CSF filled sac through a defect in the posterior elements (spina bifida). It is most typical in the lumbar or sacral regions. Although they can occur in the cervical spine. They may be anterior (usually pre-sarcral). An important point is that neural tissue is NOT present in the sac.

Answer 644

These are lipomas with a dural defect. On exam you are going to have a subcutaneous fatty mass above the gluteal crease. These are 100% associated with tethered cord (myelomeningocele may or may not).

Answer 645

This is a herniation o f a terminal | syrinx into a posterior meningocele via a posterior spinal defect

Answer 646

Most common in the thoracic spine along the dorsal aspect. | They don’t need to be (but can be) associated with posterior element defects.

Answer 647

This is often an incidental finding “fatty filum”. There will be a linear T1 bright structure in the filum terminale. The filum is not going to be unusually thickened and the conus will be normally located.

Answer 648

This is a thickened filum terminale (> 2 mm), with a low lying conus (below the inferior endplate of L2). You may have an associated terminal lipoma. The “tethered cord syndrome” is based on the clinical findings o f low back pain and leg pain plus urinary bladder dysfunction. This is the result o f stretching the cord with growth o f the canal.

Answer 649

This is an epithelial lined tract that extends from the skin to deep soft tissues (sometimes the spinal canal, sometimes a dermoid or lipoma). These are T1 low signal (relative to the background high signal from fat).

Answer 650

This describes a sagittal split in the spinal cord. They almost always occur between T9-S1, with normal cord both above and below the split. You can have two thecal sacs (or just one), and each hemi-cord has its own central canal and dorsal/ventral horns. Classification systems are based on the presence / absence o f an osseous or fibrous spur and duplication or non-duplication o f the thecal sac.

Answer 651

This is a spectrum o f defects in the caudal region that ranges from partial agenesis o f the coccyx to lumbosacral agenesis. The associations to know are VACTERL and Currarino triad. Think about this with maternal diabetes. “Blunted sharp” high terminating cord is classic, with a “shield sign” from the opposed iliac bones (no sacrum).

Answer 652

Note than the Meningocele of Currarino is Anterior. It’s not posterior. A potential deployment o f fuckery is to put “Posterior” Sacral Meningocele as a distractor for Currarino Triad.

Answer 653

``` Anterior Sacral Meningocele, • • •• • • Anorectal malformation, Sacrococcygeal osseous defect (scimitar sacrum). ```

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