Pediatric-chest, GI, GU Flashcards
1
Q
croup-what, who, syx’s, orgm
A
- acute laryngotracheobronchitis
- 6 mo-3 yrs (avg 1 yr)
- barky cough
- parainfluenza
2
Q
steeple sign
A
loss of normal shouldering/lateral convexities of subglottic trachea
3
Q
epiglottis-who, orgm cause of death
A
- 3.5 yrs (Recent spike in teenagers)
- H influenza
- death by asphyxiation from aryepiglottic folds (not epiglottis)
4
Q
mcc acute upper airway obstruction in children
A
Croup
5
Q
normal appearance of subglottic trachea
A
- “shouldering”
- lateral/outward convexities
6
Q
croup appearance on chest xray
A
- narrowing subglottic trachea or overdistanded pharynx
- epiglottis & aryepiglottic folds normal
7
Q
epiglottitis xray appearance
A
-thickening epiglottis (“thumb sign”) or aryepiglottic folds
8
Q
omega epiglottis-fake out, how to distinguish
A
- epiglottitis fake out caused by oblique imaging
- look at aryepiglottic folds
9
Q
exudative tracheitis (bacterial tracheitis)-what, orgm, who, img
A
- rare, deadly exudative infection of trachea
- staph aureus
- 6-10 yo
- “linear soft tissue filling defect in airway”, irregular tracheal plaques
10
Q
retropharyngeal cellulitis, abscess-what, causes, syx’s, who, app
A
- pyogenic infection of retropharyngeal space usually following recent pharyngitis or URI
- sudden onset fever, stiff neck, dysphagia, stridor
- 6 mo-12 mo
- massive retropharyngeal soft tissue thickening
11
Q
normal soft tissue thickness btw pst pharynx and ant vertebral body
A
- should not exceed AP diameter of cervical VBs
- ~ 6mm at C2, 22m at C6
12
Q
pseudothickening RPS-who, when, how to distinguish
A
- infants, short necks
- neck not well extended
- distinguish: true thickening=apex anterior convexity of ST, repeat with neck in full extension, gas in ST
13
Q
subglottic hemangioma-what, where, ass
A
- MC ST mass in trachea, MC subglottic
- asymmetric narrowing trachea (left MC)
- ass:
- cutaneous hemangiomas 50%
- PHACES syndromen 7%
14
Q
PHACES
A
- Posterior fossa (Dandy walker)
- Hemangiomas
- Arterial anomolies
- Coarctation of aorta, cardiac defects
- Eye abN
- Subglottic hemangiomas/Sternal Cleft/Supraumbilical raphe
15
Q
laryngeal cleft-aka, what, ass, img, dx
A
-aka laryngoesophgeal cleft
-communicating defect in pst wall of larynx and esophagus or anterior hypopharynx
- different cleft classifications
- ass malformations (usually GI, VACTER)
- fluoro: thin tract of contrast extending to larynx or trachea)
- dx=direct visualization/scope
16
Q
aw papilloma-what, cause, app
A
- lobulated mass(es) in airways & ass pulm nodules (solid & cavitated)
- HPV (perinatal transmission)
- usually mult: “papillomatosis” –> mult areas of atelectasis & air trapping (vs solitary carcinoid or FB)
17
Q
adenoids
A
- seen at 3-6 mo
- full at 1-2 yrs
- too big when encroach on airway
18
Q
How often does meconium staining of amniotic fluid occ? What does it look like? Aspiration syndrome?
A
15%, 5%
-green stained amniotic fluid
19
Q
group B Strep vs non GBS neonatal PNA
A
- GBS= mc pna, low lung vol, 67% pl eff
- non GBS-hyperinflated, patchy & asymm perihilar densities, pl eff 75%
20
Q
how often is meconium aspiration asss w/ PTX?
A
20-40%
21
Q
when does TTN start, peak and resolve?
A
6 hrs, 24 hrs, 3 d
22
Q
true or false: a normal plain film at 6 hrs excludes SDD
A
True
23
Q
Increased risks of surfactant therapy
A
- pulmonary hemorrhage
- PDA
24
Q
GBS neonatal PNA-how, who, app
A
- mc neonatal pna
- from birth canal
- (+) risk if premature
- low lung vol, granular opacities, pl effusion 25%
25
"granular opacities"
GBS neonatal PNA (pl eff) & SDD (no pl eff)
26
persistent pulmonary HTN/persistent fetal circulation-what, 2˚ causes
- persistence of high pressure in lungs
| - 1 or 2˚ (hypoxia-from pna, meconium aspiration etc.)
27
"reduced thoracic circumference" on OB US
pulmonary hypoplasia
28
"fetal lung:head ratio <1" on OB US
pulmonary hypoplasia
29
CDH associations
- cong heart dx
- malrotation (ess 100%)
- right sided -GBS
30
mortality rate in CDH related to...
degree of pulmonary hypoplasia
31
NG tube in CDH
curves into chest
32
true or false: bronchogenic cysts comm w/ aw
false
33
"recurrent PNA in same area"
intralobar sequestration
34
bronchopulmonary sequestration
extra lung that is NOT conn to aw or pulmonary arteries.
35
intralobar bronchopulmonary sequestration-how common, who, cong ass, img, where
- MC (75%)
- adolescence
- pulmonary venous drainage
- no pleural cover--> recurrent inf (bacteria migrates in from pores of Kohn)
- no ass cong anomolies
- LLL pst segm (60%), RLL 40%
36
extra lobar bronchopulmonary sequestration-how common, present, ass
- 25%, infancy
- respiratory distress/cyanosis (rel to associated congenital anomalies)
- ass w/ cong anomalies (extra for extra things)
- pleural covering--> rarely infected
- systemic venous drainage
37
Extralobar BPS associated congenital anomalies
extra for extra things:
- CCAM
- CDH
- pulm hypoplasia (Can't breath)
- cong heart disease
- vertebral anomalies (Cervical, not actually but fits the mnemonic.)
38
congenital lobar emphysema-mx
lobectomy
39
ccam-acronym
congenital cystic/pulmonary adenomatoid malformation
40
CCAM types
1) 1+ large cysts (2-10cm)-50%
2) numerous small, uniform size cysts-50%
3) solid appearing but microscopic cysts-10%
4) unlined cyst typically affecting single lobe, indist from type 1
0) acinar dysgenesis or dysplasia, represents global arrest of lung development, v rare
41
CCAM mx
- 90% spon't decrease during 3rd TM
| - resect-risk of peluropulmonary blastoma, rhabdomyosarcoma
42
mass on 2nd TM US
pulmonary congenital malformation
43
normal 2nd TM US --> mass
1˚ pulmonary tumor
44
pleuropulmonary blastoma (PPB)-what, where, img, types, ass
- Rare malignant embryonal mesenchymal neoplasm of lung & pleura that arises during organ development
- mc 2˚ lung malignancy in children. Big fucking mass in chest of 1-2 yo
- R-sided, pleural based, no chest wall invasion or Ca
- types: cystic, mixed, solid
- solid --> brain, bones
- cystic < 1yo, benign
- 10% multilocular cystic nephromas
45
PPB vs askin tumor or Ewing sarcoma of chest wall
PPB no chest wall/rib invasion
46
ideal termination of UA catheter
T8-10 or L3-L5
| avoid renal arteries
47
what abdominal cong abN is a CI to UA catheter placement
omphalocele
48
UV catheter complications
- clot in portal v--> lobar atrophy
| - cystic liver mass ==> hematoma; sugg erosion into liver
49
classic img viral pna. what is special about RSV?
- peribronchial edema
- mucus/debris in aw --> hyperinfl & subsegm atel
- RSV: segmental or lobar atel, esp RUL
50
round PNA-who, orgm, path, where, next step
-<8 yo
-S pneumonia
-path: don't have goo collateral ventilation pathways
solitary
-pst lobes
-next step: follow up xray (no CT to exclude cancer)
51
lipoid pneumonia-classic hx, app, dx test of choice?
- parents giving olive oil daily --> chronic fat aspiration
- low attenuation in consolidated areas
- dx=bronchoalveolar lavage?
52
Swyer James-which lung is abnormal?
- post viral obliterative bronchiolitis --> affected lung smaller than normal/hyperexpanded lung.
* cause of unilateral Lucent lung
53
pulmonary hypoplasia
lung tiny or incompletely developed-1˚ or 2˚
54
pulmonary papillomatosis-app, risk/compl, sim to what disease?
- ST masses in aw & lungs/"multiple lung nodules w/ cavitation
- 2˚ risk SCC
- cysts & nodules = LCH + tracheal involvement
55
sickle cell/acute chest
- kids > adults (2-4)
- leading cause of death
- rib infarct --> pain causes decreased inspiration --> atelectasis/ infection
- pulmonary microvascular occlusion, infarct
- img: opacities
56
findings of sickle cell in cheset
- big heart
- humeral head infarcts
- h shaped vertebra
- cholecystectomy clips
57
CF in the chest
- apical predominant
- mucous plugging --> bronchiectasis, hyperinflation, finger in glove
- PAH
58
primary ciliary dyskinesia
- ciliopathy-motile part doesn't work
- lower lobe predominant bronchiectasis
- kartageners 50%
- infertility (M); sub fertility (F)
59
why are men infertile in CF vs 1˚ ciliary dyskinesia
- CF: vas deferences absent
| - PCD: sperm don't swim
60
mc mediastinal mass
thymus
61
sail sign vs spinnaker sail sign of thymus
sail sign=normal triangular shape of thymus in kids (esp large in <5 yo)
-spinnaker sail sgx-lifting of thymus in PMX
62
things that make you think thymus cancer
- abN size for age (~15 yo)
- heterog
- Ca
- compression aw/vasc
63
thymic rebound
thymus shrinks in acute stress (PNA, radiation, cry, burns)
- recovery phase= return to size (sometimes larger); pet+!
- grows but maintains N triangular morphology
64
mc abnormal mediastinal mass in children
lymphoma (~>10 yrs vs normal thymus)
65
lymphoma vs ALL/leukemia
indistinguishable on img (st mediastinal mass)
66
47 XXY
klinefelter's syndrome
67
3 flavors of mediastinal GCT
- teratoma-cystic, fat, Ca
- seminoma-midline; bulky, lobulated
- NSGCT- big & ugly; hemorrhage, necrosis; lung invasion
68
who's at increased risk chest GCTs?
Klinefelter's syndrome (300x risk)
69
middle mediastinal LAD causes
- granulomatous (Tb or fungal) MC; lymphoma
- mono (EBV 90%, CMV 10%)
- 1˚ tb
- histoplastmosis
- coccidioidomycosis
- lymphoma
- sarcoid
70
what do pt's with mono get after amoxicillin
rash
71
histoplasmosis-US region, chest imaging
- MW, SE
| - CXR: N (MC), hilar LAD
72
coccidioidomycosis-US region, chest img
-SW
-consolidation, nodules
hilar LAD
73
mediastinal lymphoma-which type
Hodgkins (4x MC than NHL)
| -HL involves thymus 90%
74
pediatric chest sarcoid
- rare, onset usually ~20s
| - few case reports
75
mediastinal duplications cysts-
- bronchogenic
- enteric
- neuroenteric
76
enteric mediastinal duplication cyst
- abut esophagus, same attenuation, may comm (fluid/air)
- distal R esophagus=2nd MC loc (ileum #1)
- middle or pst mediastinum
77
bronchogenic mediastinal duplication cyst
- abut trachea or bronchus, NO comm
- fluid/mucous filled
- subcarinal
- middle mediastinum (70%), hila (30%)
78
pst mediastinal masses-peds
- NB. GN, GB
- Ewing Sarcoma
- Askin Tumor (primitive neuroectodermal tumor of cw)
- neuroenteric cyst
- extramedullary hematopoiesis
- round PNA
- extralobar sequestration
79
neuroblastoma vs ganglioneuromas
- GN: less aggressive, more cirdcumscribed, no Ca, older children
* can't differentiate based on imaging alone)
80
askin tumor (primitive neuroectodermal tumor of chest wall)-aka, features/img
part of Ewing sarcoma spectrum!
- aka "Ewing sarcoma of cw"
- displace rather than invade (exc when large)
- heterogenous, enhancing solid parts, no Ca
81
neuroenteric cyst-where, features, ass
Cyst along neuraxis (usually intraspinal)
- Most are intradural, extramedullary simple unilocular cysts ventral to spinal cord
- lower cervical/thoracic region
- well-demarcated, fluid att. no comm w/ csf
- ± vertebral anomalies, other closed spinal dysraphisms
82
extra medullary hematopoiesis-who, app, ass
myeloproliferative disorders, bone marrow infiltration (including SCD)
- BL, smooth sharply delineated ST paraspinal masses
- + HMG, SMG
83
mc posterior mediastinal mass in child <2 yo
neuroblastoma
84
what does better: thoracic or abdominal neuroblastoma
Thoracic
85
imaging pst mediastinal NB
- pst mediastinal st mass, heterog + enh, Ca
| - +/0 invasion ribs, VBs
86
spectrum ganglioneuromas
- ganglioneuroma (most mature)
- ganglioneuroblastoma (intermediate)
- neuroblastoma (undifferentiated)
87
normal thymus vs lymphoma?
age: <10 yo n thymus, >10 yo lymphoma
88
anterior mediastinal masses-peds
-normal thymus
-lymphoma
thymic hyperplasia
GCT
lymphangioma
89
mediastinal lymphoma-img
- usually >10 yo
- ant or middle mediastinal mass
- thymus, homogenous ST density, pericardial effusion, vascular/airway involvement
- Ca uncommon if untreated
90
pst mediastinal masses-approach
<10 yo: malignant; NB
>10 you: benign:
-round mass: ganglioneuroma, NF
-cystic + spinal anomaly: neuroenteric cyst
-course bone trabecularion, hx anemia-EMH
91
big chest masses
Askin (PNET/Ewings)
Pleuopulmonary blastoma
*age
92
MC TE fistula
N type (85%)
93
esophageal atresias/fistulas-types to know
- N type (85%)=blind ending esophagus w/ distal TE fistula
- Esophageal atresia, no fistula (10%)
- H type (1%)=no atresia, TE fistula; delayed diagnosis
94
TE fistulas w/ excessive air in stomach
N and H types
95
fake out for TE fistula
simple aspiration (look for laryngeal penetration)
96
what must be described prior to surgery in setting of EA/TE
right arch (4%)-changes approach
97
VACTERL
```
3+ anomalies. Heart & kidney MC
Vertebral (37%)
Anal (63%)
Cardiac (77%)
TE (40%)
Renal (72%)
Limb (radial ray) (58%)
```
98
relationship btw limbs and kidneys in VACTERL
- Both limbs involved --> both kidneys
| - One limb involved --> one kidney
99
FB in trachea vs esophagus
- esophagus flexible, accommodates FB-coin turned in any dir
| - trachea rigid, forces coin to rotate into pst mem (skinny in AP dir)
100
FB, what to do: AA or AAA batteries
- serial xray
| - remove if in stomach >2d
101
FB, what to do: disc batteries
- es-2 hrs
| - stom-4 hrs
102
disc battery vs coin
disc=2 rings
103
FB, what to do: coin
remove if:
- es 24 hrs
- stom 28 d
104
FB, what to do: penny minted after 1982
remove from stomach
105
what makes pennies minted after 1982 special? what's the problem?
- contain zinc
- problems:
- + stomach acid--> gastric ulceration
- Zn toxicosis (if absorbed in great enough quality)--> pancreatic dysfunction, pancreatitis
106
FB, what to do: lead
- remove immediately from stomach (gastric acid leads to immediate absorption)
- distal passage must be confirmed
107
FB, what to do: sharp objects
- esophagus-remove immediately
- stomach-removed immediately
- post pylorus=follow vs sx (if it does not perforated small bowel, it will be at IC valve)
108
classic ddx obstruction in older child-AIM
- appendicitis; adhesions
- inguinal hernia; intussuscpetion
- midgut volvulus; meckels
109
vascular rings & slings
- Pulmonary sling
- double aortic arch
- Innominate artery compression
- aberrhant left subclavian + right arch
- aberrhant right subclavian + L arch
110
only vascular ring/sling to go btw es & trachea?
pulmonary sling
111
what tracheal abnormality is pulmonary sling ass with?
tracheal stenosis
112
pulmonary sling is ass with many other cardiopulmonary & systemic anomalies. which ones?
hypoplastic right lung, horseshoe lung, TEF, imperforate anus, complete tracheal rings
113
pulmonary sling rx
surgical repositioning of artery (controversial)
114
dysphagia lusoria
trouble swallowing in setting of left arch with aberrant right subclavian
115
diverticulum of kommerell
pouch like aneurysmal dilation of proximal portion right subclavian artery
116
finding highly specific for duodenal atresia? when would you see it?
- double bubble
| - 3rd TM OB US, plain film or MRI
117
what is malrotation ass with?
- heterodoxy syndromes
- omphalocele
* of note: SMA to right of SMV
118
FP malrotation on upper GI
distal bowel obstruction displacing duodenum (bc of ligamentous laxity)
119
corkscrew duodenum
midgut volvulus
120
Next step: non-bilious vs bilious vomiting
- US (pyloric stenosis)
| - upper GI (midgut volvulus until proven otherwise)
121
Ladd's bands
fibrous stalk of peritoneal tissues that fixes cecum to abdominal wall --> obstruction duodenum (intermittent episodes)
122
partial vs complete duodenal obstruction
- complete= midgut volvulus, duodenal atresia
| - partial=ladd band, annular pancreas, duodenal web, stenosis
123
pre-duodenal portal vein-what, ass
anatomic variant, PV sit ant to D2
| -duo obstr 50%, but ISN'T the cause (ass w/ other things...ladds bands, annular pancreas, etc)
124
Borchardt Triad
Gastric volvulus
1) inability to pass NGT
2) severe epigastric pain
3) retching w/o vomiting
125
When does hypertrophic pyloric stenosis occur?
specifically 2-12 wks (ie: not at birth and not >3mo)
| -peak-3-6wks
126
primary differential pyloric stenosis
pyorospasm (will relax during exam)
127
MC patient pitfall during exam for pyloric stenosis that creates FN?
gastric overdistention --> displacement antrum & pylorus --> FN
128
pylori stenosis FP
off axis measurement
129
"paradoxical aciduria"
pyloric stenosis
130
Organoaxial vs mesenteroaxial age diff
Organo=Old
131
duodenal atresia association
- DS (30%)
- other intestinal atresia
- biliary abN
- cong heart dx
- VACTER
132
How do we know that double bubble is not an acute obstruction caused by a midgut volvulus, which is, a surgical emergency?
Dilatation of the duodenal bulb is seen only with chronic causes of obstruction. Not enough time for the bulb to become dilated in acute obstruction, such as with midgut volvulus
133
duodenal atresia vs stenosis
- atresia=occlusion, stenosis=fixed narrowing
| - With duodenal stenosis, the double bubble is seen in association with the presence of distal bowel gas
134
duodenal web-what, where, ass, img
- partial canalization (not total like atresia)
- distal to ampulla
- ass: DS, malrot
- pin sized hole;
- wind sock deformity-older kids where web-like diaph stretched
135
small left colon- aka, what, mx
- meconium plug
- Functional colonic obstr,
- self limited, relieved by contrast enema
136
What is small left colon ass and NOT ass with
- NOT ass w/ CF
| - mothers with DM or Mg sulfate for eclampsia
137
Hirschsprung dx-who
boys (4:1)
| DS
138
HD dx imaging
- recto-sigmoid ration <1
| - saw tooth (bowel spasm)
139
HD presentation
- no BM > 48-72 hrs
- forceful passage meconium after rectal exam
- nec bowel s/p 1 mo
140
total colonic aganglionosis
rare variant of HS mimicking microcolon
| *can involve TI
141
calcified mass in mid abd
- meconium peritonitis. Sterile peritoneal rxn to in-utero bowel perforation ( w/ bowel atresia or MI
- perforation seals
142
imperforate or ectopic anus-range, ass
- anal atresia --> arrest of colon as it descends through pubrectalis sling
- fistula to GU tract
- ass=VACTERL, tethered cord-need screening US
143
MCC bowel obstruction peds > 4yo
appendicitis
144
mcc obstruction in boy 1mo-1yr
indirect ing hernia
145
when does intuss present?
3mo-3 yr, no lead points
146
when does intuss matter?
>2.5 cm
| -SB-SB <2 and spon't reduce
147
intuss img
target sign
| pseudo kidney
148
suspected lead points for intuss
HSP, MD, enteric cysts
149
CI enema reduction
- free air
- peritonitis
- septic/hypovolemic shock
150
Intuss success rate, recurrence timeframe, risk perforation (what to do?)
- succ: 80-90% (reduced with HSP)
- recurr 72 hrs
- 0.5% perforation --> needle decompression. *air causes less peritonitis than barium"
151
Meckel's diverticiulum
congenital diverticulum of distal ileum, persistent piece of omphalomesenteric duct
152
MD rule of 2's
- 2% population
- 2 types of heterotypic mucosa (gastric, pancreatic)
- 2 ft of IC valve
153
complications MD
- diverticulitis
- bleed (30%)
- intuss (seen w/ inverted diverticulum)
- obstruction
154
pentology of cantrell
1) omphalocele
2) ectopic cordis (abN location of heart)
3) diaphgragmatic defect
4) pericardial defect or sternal cleft
5) cardiovascular malformation
155
right sided extra-abdominal evisceration of neonatal bowel and sometimes stomach/liver through paraumbilical wall defect
gastroschisis
156
gastroschisis ass
intestinal atresia
157
gastroschisis maternal labs
AFP+ (higher than omphalocele)
158
complication gastroschisis repair
reflux
159
omphalocele ass
Trisomy 18 MC chrom
cardiac (50%)
syndromes-Klinefelter, BW, Turners, Pentalogy of Cantrell
160
probably causes gastroschisis vs omphalocele
- gastroschisis-environmental (ass w/ bowel atresia)
| - genetic-ass w/ various other syndromes
161
enteric duplication cysts-what, compl, ass
- development anomalies (failures to canalize), can comm with lumen (but usually don't)
- can obstr, perf
- ass: vertebral anomalies (30%)
162
cyst w/ gut signature
enteric duplication cyst
163
cyst w/o gut signature
omental cyst (lymphangioma=MC)
164
necrotizing enterocolitis-path
immature bowel mucosa --> translocation bacteria --> ischemia & inf
165
who gets NEC
- premature (90% w/I 1st 10 days)
- LBW (<1500 g)
- cardiac-can be FT
- perinatal asphyxia
- Hirschsprung kids that go home and come back
166
NEC findings
- pneumatosis (most definitive), PV gas (2nd most)
- focally dil bowel (RLQ)
- featureless small bowel w/ separation
- unchanging bowel gas pattern, ie: several plane films w/ bowel gas pattern unchanged.
167
where does NEC mostly occur?
right colon/terminal ileum
168
what is only parameter ass w/ decreased NEC
maternal breast milk
169
what do you ask yourself in determining NEC vs feces?
first-did the kid eat?
| secondly- is it moving?
170
how often is pancreas involved in CF?
90%
171
dorsal pancreatic agenesis-what, ass
- dorsal bud does not form --> absent tail --> DM (most beta cells are in tail)
- ass w/ polysplenia
172
peds pancreatic mass: age 1, 6, 15
pancreatoblastoma, adenoCA, SPEN (solid & papillary epithelial neoplasm)
173
mc pediatric pancreatic solid tumor
Solid and papillary epithelial neoplasm (SPEN)
- female adolescent (asian or black)
- rx-surgical (good outcome)
174
pediatric liver tumors ages 0-3 yrs
- infantile hepatic hemangioma
- hepatoblastoma
- mesenchymal hamartoma
175
infantile hepatic hemangioma-ass findings, syndromes, labs
- liver mass + CMG (high output CHF)
- aorta above hepatic branches enlarged relative to aorta below celiac via differential flow
- +skin hemangiomas 50%
- endothelial GF+
- kasabach-merritt Syndrome (platelet eater)
- spon't involute w/o therapy mo's-yrs, prog Ca+
Hemangioma: Benign endothelial neoplasm of neonates/infants in soft tissues or viscera (especially liver)
- Not hemangioendothelioma (more aggressive tumor)
- Not cavernous hemangioma (venous malformation)
```
Congenital hemangioma (CH): Found in perinatal period, does not proliferate beyond birth; stains GLUT1 negative
Rapidly involuting subtype more common in liver than noninvoluting
```
Infantile hemangioma (IH): Develops in 1st few weeks of life with characteristic proliferating & involuting phases; stains GLUT1 positive
176
hepatoblastoma-app, labs, ass, RF
-well circumscribed solitary RIGHT sided mass, Ca 50%, +/- ext into PV, HV, IVC
-AFP+
bHCG--> precocious puberty
-ass: hemi-HTr, *Wilms, BWd
-RF: prematurity
177
mc 1˚ liver tumor of childhood <5yo
hepatoblastoma
178
mesenchymal hamartoma
- Cystic. Large portal v branch feeds tumor. solid comp mildly enh
- AFP-
- ø Ca
179
pediatric liver tumors > 5yrs
- HCC
- fibrolamellar
- undifferentiated embryonal sarcoma
* young children/teens: hepatic adenoma, hemangioma, FNH, angiosarcoma
180
2nd mc liver cancer in kids
HCC
181
undifferentiated embryonal sarcoma-what, app
- pissed off cousin of mesenchymal hamartoma, ie: cystic but much more aggressive
- hypodense +sept & fibrous pseudocapsule
* known to rupture
182
pediatric liver tumors w/ elevated AFP
- hepatoblastoma
| - HCC
183
what liver tumors would you expect at any age?
-mets (Wilms, NB)
184
fetal HMG
- congenital syphilis
| - transient abN myelopoiesis (TAM)
185
congenital syphilis- findings, when, earliest and last orgn
- prenatal US >20wks: HMG + enlarged placenta
| - liver earliest and last
186
transient abN myelopoiesis (TAM)-what, who, mx
preleukemic syndrome in DS
- advanced maternal age
- 80% self-resolve; 20% --> myeloid leukemia
187
hereditary hemorrhagic telangiectasia (osler-weber-rendu) in liver
- mult AVMs
- dilated hepatic a
- cirrhosis
188
prolonged newborn jaundice (>2wks)
1) neonatal hepatitis
| 2) biliary atresia
189
mx biliary atresia
Kasai procedure (bf 3 mo)
190
biliary atresia-findings, ass, test of choice, mx
- absent extra hepatic biliary ducts --> proliferation intrhepatic
- +/- absent gb (normal GB supports neonatal hep)
- triangle cord sgx
- nuc medicine IDA = test of choice
- ass-polysplenia, trisomy 18
- mx
- bx-exclude alagille
- kasai procedure (bf 3 mo)
191
alagille syndrome
- congenital genetic multisystem disorder. Infants typically present with symptoms relating to the liver where it is one of the most common causes of hereditary cholestasis.
- hereditary cholestasis (from paucity intrahepatic bd's)
- peripheral pulmonary stenosis
192
what is the purpose of liver bx in biliary atresia?
exclude alagille syndrome
193
triangle cord sign
triangular echogenic structure near portal v in biliary atresia
-possibly CBD remnant?
194
peds + gs
sickle cell
195
situs solitus
normal: left gastric bubble, larger part of liver on right, minor fissure on R
196
situs inversus totalis-what, findings, ass
total mirror image transposition of abdominal and thoracic contents
- gastric bubble on right
- large liver on left
- left minor fissure
- inverted bronchial pattern
- ass: 1˚ ciliary dyskinesia
197
situs ambiguus
"heterotaxy", ie: L or R isomerism
198
changes of spleen in sickle cell
-enlarge progressively --> auto infarct & shrink (1st decade) (painless)
199
what can happen if sickle cell spleen remains enlarged? Hx?
1) acute splenic sequestration crisis-hogs blood; 2nd mcc death SCC
- hx=abd pain, hypotension and SMG
2) abscess
3) infarct
200
how does spleen infarct look on us?
hypoechoic + bright bands
201
heterotaxia syndromes
Right vs left sided
202
Right sided heterotaxia syndrome/Right isomerism
- 2 left pleural fissures
- asplenia
- more cardiac malform
- reversed aorta/ivc
203
Left sided heterotaxia syndrome/left isomerism-findings, ass
- Absent minor fissures (1 right fissure)
- polysplenia
- fewer cardiac malform
- azygos con't of IVC
- ass: biliary atresia (10%)
204
renal agenesis 2 flavors & ass
1) BL absence- Potter sequence
| 2) UL abscence-reproductive
205
how will they demonstrated UL renal agenesis on prenatal US?
absent renal artery in view of aorta OR oligohydramnios
206
UL renal agenesis associations
- 70% F-unicornuate uterus or rudimentary horn
| - 20% M-absent epididymis, IL vd + IL seminal vesicle cyst.
207
potter sequence:
insult (?ace inh)--> renal agenesis --> oligohydramnios --> pulm hypoplasia
208
lying down adrenal or "pancake adrenal" sgx
elongated adrenal in renal agenesis (vs surgical absence)
209
MC kidney fusion anomaly
horseshoe kidney (hung up by IMA)
210
horseshoe kidney complications
1) prox to VB--> injury
| 2) poor drainage-stones, inf, CA (chr inflamm) (Wilms, TCC, renal carcinoid)
211
classic horseshoe kidney association
turner's syndrome
212
crossed fused renal ectopia-what, which side MC, compl
- one kidney crosses midline & fuses with other. Each maintains orthotropic ureteral orifice.
- ectopic kidney is inf
- L>R
- compl: stones, inf, hydro (50%)
213
how will exam show crossed fused renal ectopia
-2 axial CT slices-1 at top without kidney, 1 at bladder with 2 opacified ureters
214
MC congenital anomaly of GU tract in neonates
congenital UPJ obstruction
215
congenital UPJ obstruction-how often BL, mech, app, rx, role of radiologist, classic history
- 80% intrinsic defect in circular m bundle of renal pelvis
- hx: teenager with flank pain after drinking lots of fluids
- hydro w/o hydroureter, BL 20%
- look for vessels crossing UPJ (changes mx)
- rx- pyeloplasty
216
Whitaker test
urodynamics study + antegrade pyelogram
217
ARPKD liver involvement -what's involved, how is it diff from AD, compl
- abN bd and congenital hepatic fibrosis (vs cysts in AD)
- inverse rel btw liver & kidney inv
- portal HTN --> death (usually MCC death)
218
ARPKD img
- smoothly enlarged, diffusely echogenic
- loss of corticomedullary differentiation
- cysts=tumular, spare cortex
- +/- in utero ø urine in bladder
219
ARPKD compls
- htn
- renal fx
- portal htn --> death
220
neonatal renal v thrombosis- path, app/pres
- maternal diabetes, sepsis, dehydration; peripheral --> hilar
- L>R
- acute=renal enlargement
- chronic=atrophy
221
neonatal renal artery thrombosis-cause, pres
UA catheters
| -severe HTN
222
prune belly (eagle barrett syndrome)-who, what, app
- males shaped like a pear
- triad:
1) ø abd wall m
2) hydroureteronephrosis
3) cryptorchidism
223
congenital (primary) megaureter
- wastebasket term for enlarged ureter intrinsic to ureter (ie: not 2/2 distal obstr)
- 3 causes:
1) distal adynamic segm
2) reflux at UVJ
3) idiopathic
224
congenital/1˚ megaureter vs obstruction
obstruction: collecting system dilated
225
retrocaval (circumcaval) ureter- what, classic six's, syx's
- anomaly of IVC. Proximal ureter courses pst to IVC, emerges to right of aorta and ant to R iliac vessels
- reverse J or fishhook ureter
- asyx (MC), obstruction, recurrent UTI
226
Weigert Meyer Rule
upper pole inserts inf and medially
227
kidney size duplicated system
large
228
compl duplicated system in F
incontinence (ureter may insert below sphincter into vagina
229
ureterocele-path, classic sign, ass
- cystic dilation intravesicular ureter 2/2 obstruction at ureteral orifice
- "cobra head sign"-on IVP or US
- ass: upper pole moiety in dupl system
230
ectopic ureter-insertion and syx
- F: usually distal to external sphincter in vestibule
| - M: usually above external sphincter --> asyx
231
mcc ureteral obstruction in male infants
pst urethral valve
232
org of pst urethral valve fold
wolfian duct
233
pre-natal US classic triad Pst urethral valve
- hydronephrosis
- bladder dilation
- oligiohydramnios
234
"key hole"
-appearance of bladder on fetal MR in pst urethral valve
235
pst urethral valve img
- VCUG-abrupt caliber change, dilated pst urethra, normal ant urethra
- fetal MR- hydro + key hold
- prenatal US: hydro, bladder dilation, oligiohydramnios
236
"peri-renal fluid collection"
2/2 forniceal rupture
- non spec, seen with any obstructive path
- ?classically pst urethral valve
237
non-obstructive causes of hydro in baby boys
- VUR
- 1˚ megaureter
- prune belly
238
obstructive causes of hydro in baby boys
- PUV
- UPJ Obstr
- ureteral ectopia
239
next step: hydro on routine prenatal screen:
repeat US once born
- most go away
- if persistent...
1) VCUG (GS),
2) MAG3 (function & drainage),
3) MR urography (function, structure; sedation required.
240
mcc palpable renal mass in childhood
VUR
241
Vascular complication PUV
htn
242
grading VUR
1) ureter
2) ureter + non dilated CS (calyces still pointy)
3) ureter + mild dil (calyces blunted)
4) ureter + mod dil
5) tortuous
243
hutch diverticulum-what, dx, rx
- congenital muscular defect at or just above UVJ. Congenital bladder diverticula, seen at the vesicoureteric junction, in the absence of posterior urethral valves or neurogenic bladder. They are thought to result from a weakness in the detrusor muscle anterolateral to the ureteral orifice.
- dx: VCUG>US. Dynamic (voiding)
- rx-VUR
244
allantois
umbilical attachment to bladder
245
Complication of urachal remnant
infection (MC)
| -adenoCA 90%
246
spectrum urachal remnant anomalies
patent, vesiculourachal diverticulum, urachal cyst, umbilical urachal sinus
247
bladder exstrophy-what, img (classic sign), compl
- herniation urinary bladder through hole in ant infra-umbilical abd wall
- "manta ray sign"-unfused pubic bones
- compl: adenoCa
248
cloacal malformation
GU & GI drain into common opening
| -F
249
cloacal malformations and urachal anomolies: F or M?
urachal: M 2x > F
cloacal: only F
250
neurogenic bladder ass
spinal dysraphism: tethered cord, sacral agenesis, etc.
251
pediatric renal masses: neonate, ~4yo, teen
- neonate: nephroblastomatosis, mesoblastic nephroma
- ~4: Wilms, Wilms variant, lymphoma, multilocular cystic nephroma
- teen: lymphoma, rcc
252
"solid renal tumor of infancy"-you can be born with it
mesoblastic nephroma
253
nephroblastic rests
leftover embryologic renal tissue
254
"solid renal tumor of childhood"-never born with it
wilms
255
renal + brain tumor in infancy
rhabdoid
256
multilocular cystic nephroma app
- big cysts that don't communicate
| - septal enhancement
257
what can't you distinguish multi cystic nephroma from?
cystic wilms-must resect
258
solid renal tumor of adolescence
rcc
259
"Michael Jackson tumor"
multi cystic nephroma
260
What is the MC RCC subtype?-img, classic hx
"translocation Carcinoma RCC"
- dense solid, enh & expansile growth
- hx prior cytotoxic crx
261
RCC subtype: sickle cell trait
medullary subtype
262
RCC subtype: VHL
Clear cell subtype, often BL
263
necrosis in nephroblastomatosis should make you think...
wilms
264
how often do you US screen nephroblastomatosis?
q3 mo until 7-8 yo
265
mesoblastic nephroma: underlying path, MC age, where in kidney, img ass
- fetal hamartoma
- 80% first mo
- renal sinus
- antenatal US: +/- polyhydramnios
266
multi cystic dyspastic kidney
- multiple tiny cysts form in utero. Macro cysts on img.
- no functioning renal tissue
- CL renal tract aN 50% (mc UPJ obstr)
267
syndromes ass with wilms
- beckwith-wiedemann (overgrowth)
- Sotos (overgrowth)
- WAGR
- Drash
268
Beckwith-Wiedemann
- macroglossia (MC)
- omphalocele
- hepatoblastoma
- wilms
- hemiHTr
- Cardiac
- big orgns
269
Sotos syndrome
macrocephaly
Retarded
Ugly face
270
WAGR
wilms
aniridia
genital
growth retardation
271
Drash
wilms
pseudohermaphroditism
progresive glomerulonephritis
272
synchronous bilateral Wilms
BL in 5-10%
273
wilms age range
- avg 3 yo
- all before 10 yo
- never before 2mo
274
should you bx wilms?
no-seeding of tract --> upstage
275
wilm's variants
clear cell--> bones (lytic)
| rhabdoid- brain tumors
276
multilocular cystic nephroma
- noncommunicating fluid filled located surround by thick fibrous capsule
- by definition, no solid comp or necrosis
- bimodal
- "protrudes into renal sinus"
277
renal rumor that "protrudes into renal sinus"
multilocular cystic nephroma
278
2nd mc renal malignancy of childhood
RCC (MC translocation carcinoma)
279
RCC (MC translocation carcinoma)
- hx: prior cytotoxic crx
- denser than cortex on non-con CT
- solid, enh, expansile growth
280
RCC vs WIlms
RCC smaller, older color, more likely to Ca
281
renal lymphoma
never 1˚
| BL expansile masses
282
initial imaging bladder masses
US w/ full bladder
283
MC pediatric tumor of bladder
rhabdomyosarcoma (cause obstr)
284
Rhabdomyosarcoma botryoid subtype img app
"bunch of grapes"-multiple round masses grouped together,
285
rhabdomyosarcoma locations
- H/N (orbit, NP)=MC
| - 20% bladder, prostate
286
how often is RMS met at pres?
20%
287
RMS bimodal peak
2-4yo, 15-17yo
288
RMS non-botryoid subtype img app
big nodular ugly horrible nightmare
289
mc extratesticular mass in young men
RMS
290
MC extra-cranial solid childhood malignancy
neuroblastoma
291
neuroblastoma locations
adrenal 35%,
RP 30%,
pst mediastinum 20%,
neck 5%
292
NB age range
<2yo (can be born with it)
| -95% <10 yo
293
what upstages NB?
stage 3:
- crossing midline
- CL positive nodes
294
NB better prognostic factors
- <1yo
- thoracic primary
- stage 4S
295
stage 4S high yield:-who, met locs, prog?
<1 yo
- mets confined to skin, liver, BMARROW (not cortical bone!) (lytic)
- excellent prognosis
296
NB ass
NF1, HSD, DiGeorge BW
| *most are sporadic
297
opsomyoclonus
- dancing eyes & feet
| - PNS ass w/ NB
298
best nuc medicine scan for NB?
-MIBG
299
NB labs
urine catecholamines+ (95%)
300
NB vs Wilms
- NB <2yo, poorly marginated, Ca 90%, encases vess, bone met
| - Wilms: ~4 yo, well marginated, Ca <10%, invade vess, no bone met (prefer lungs)
301
neonatal adrenal hemorrhage cause and ass
- birth trauma, stress
| - ass: scrotal hemorrhage
302
adrenal hemorrhage vs NB. First step.
- hem: anechoic, T2(-), avascular
- NB: hyper echoic, T2(+), hypervascular
- first step: f/u US
303
mcc adrenal insufficiency in kids?
congenital adrenal hyperplasia 2/2 21 alpha hydroxyls deficiency
304
CAH img
cerebriform adrenals
305
hydrometrocolpos-what, causes, pres, app, ass
- obstructed vagina does not drain uterus
- causes-imperforate hymen (MC), vaginal stenosis, lower vaginal atresia, cervical stenosis
- pres: infancy-mass or teenage-delayed menarche
- expanded, fluid/blood-filled vaginal cavity w/ distention of uterus
- ass: uterus didelphys (75% have transverse vaginal septum)
306
ovarian torsion peds vs adult
- adult-mass
| - peds: normal excessive mobility. "fluid-debris levels within displaced follicles"
307
suspect ovarian torsion if...
-ovary 3x size CL
| + fluid-debris levels in displaced follicles
308
ovarian pediatric masses
- 2/3 dermoid/teratomas-MC
| - 1/3 cancer (75% germ cell tumor)
309
mcc idiopathic scrotal edema
HSP
310
pathology hydrocele
patent processus vaginalis
311
pediatric acute scrotal pain: 3 considerations
1) testicular appendage torsion
2) testicular torsion
3) epididymo-orchitis (isolated orchitis is rare)
312
age range epididymitis
<2 yo, > 6yo
313
causes of orchitis
- nearly always progress from epididymitis
| - isolated=mumps
314
testicular appendage
vestigial remnant of mesonephric duct
315
blue dot sign
physical exam sign in testicular appendage torsion. (testicle looks like blue dot)
316
best indicator testicular appendage torsion?
>5mm
317
bell clapper deformity
failure of tunica vaginalis and testis to connect
318
histologic breakdown of testicular cancer (adult & peds)
```
germ cell 90%
-seminoma 40%
-non-seminoma 60%- Teratoma, YS, Mixed GC (chorioCA)
Non GC (10%)
Leydig
Sertoli
```
319
Germ cell tumors seen in 1st decade of life
YS (heterogeneous appearance) & teratoma
320
teratomas M vs F
aggressive in men
321
sertoli cell tumors ass
subtype associated with peutz-jeghers syndrome
322
testicular calcifications and CA
small=seminoma
| big=GCT
323
mc tumor of fetus or infant
sacrococcygeal teratoma
324
sacrococcygeal teratoma compl
- benign (80%); older infant pres=higher malignant pot
| - large size --> ME on GI system, hip dislocation, n compression--> incontinent
325
sacrococcygeal location
- external to pelvis (47%)
- internal to pelvis (9%)
- mixed (dumbbell)-34%
326
what's associated with high sacrococcygeal recurrence rate
incomplete resection of coccyx
327
sacrococcygeal classification. Which is ass w/ highest malignancy rate?
type 1- totally extra pelvic
type 2-barely pelvic but not abd
type 3- some abd
type 4-totally abd. Highest mal.
328
testicular tumors/masses to consider
- Germ cell tumors-teratoma, YS. Others (embryonal, chorio, seminomatous, mixed) more rare in peds
- Epidermoid
- Choriocarcinoma
- Lymphoma/leukemia
- adrenal rests
- abscess
- infarct
329
pediatric abdominal calcification
- meconium peritonitis
- neo-NB, teratoma, HB
- adrenal hemorrhage
- RUQ: gs, HB, hepatic torch (CMV, toxo)
330
hepatic infantile hemangioma/hemangioendothelioma vs other hemangiomas
It's a highflow vascular neoplasm. "hemangioma" otherwise is venous malformation
331
hepatic infantile hemangioma vs hemangioendothelioma
- both: benign high flow vasc neo's. CHF
| - diffs: HE can metastasize
332
hepatic infantile hemangioma- ass, mx
- Kasabach-Merrit syndrome & GLUT-1
| - b-blockers, sx if CHF
333
pro ass with infantile hemangioms
GLUT-1
334
img hepatic infantile hemangioma
focal, MF, diffuse.
- vascular
- T2+
- enh peripherally w/ delayed fill in
- Ca, central necrosis, hemorrhage
335
clear cell sarcoma
"bone loving tumor"
| -uncommon renal cancer with propensity to metastasize to bone
336
pediatric renal met
NB, L/L
337
congenital lobar overinflation/hyperinflation/emphysema-moa
one way valve
338
renal tubular ectasia-aka
ARPKD. Newborn BL kidney enlargement, echogenicity, reversal/loss of normal CM diff (ie: medulla hyperecho) via dilated ectatic tubules
339
newborn kidney appearance on ultrasound
The US appearance of the kidneys in neonates and infants up to 6 months of age is distinctive and differs significantly from that in older children and adults. The renal cortex in neonates has echogenicity equal to or greater than that of the liver and spleen, whereas in older children and adults, the cortex is hypoechoic relative to those organs. The echogenicity of neonatal renal cortex is due to the relative concentration, as well as the increased cellular volume, of glomeruli. The medullary pyramids appear prominent and hypoechoic because of a relatively lower cortical volume (,Fig 1). By the time a child is 6 months old, the echogenicity of the renal parenchyma assumes an adult pattern. In addition, the intense echogenicity due to fat in the renal sinuses of older patients is relatively scant or absent in the newborn infant (,4).
340
acute scrotum-order of frequency
EO
appendage torsion
testicular torsion-most serious/needs tromp dx
341
aniridia
absent iris
342
pleuroblastoma cystic vs solid types
- solid --> brain, bones
| - cystic < 1yo, benign
343
pulmonary agenesis vs aplasia vs hypoplasia
Agenesis: Absent lung, pulmonary artery, & bronchus
Aplasia: Absent lung & pulmonary artery with rudimentary blind-ending bronchus
Hypoplasia: Hypoplastic lung, pulmonary artery, & bronchus
