HomeStretch CRACK vol 1. PEDS Flashcards
Trigonocephaly
Metopic synostosis
“quizzical eye appearance”
Brachycephaly
Coronal synostosis
“Harlequin eye” if unilateral
Turricephaly
(bilateral) lambdoid synostosis
Sagittal synostosis
Scaphocephaly
Dolecocephaly
“scaphodolecocephaly”
(associated with Marfans [both are tall and Skinny).
When I say “bone defect in the region of the lambdoid suture” or “asterion defect” you say?
NF1
Kleeblattschädel
clover leaf skull
premature synostosis of coronal and lambdoid sutures +/- sagittal suture
assocaciated with: thanatophoric dysplasia, Apert and crouzon’s
Apert Syndrome
Craniosynostosis (brachycephaly) + Fused fingers aka syndactyly (making an “A”) - typically symmetric fused hands and feet.
Crouzon’s
Craniosynostosis (brachycephaly)
1st arch structures (maxillary and mandibular hypoplasia)
Hydrocephalus
Chiari 1 malformations
associated with patent ductus and aortic coarctation
short central long bones (rhizomelia)
Crouzon’s Cs = Coronal suture craniosynostosis, Can’t Chew (first arch structures), Chiari I, Coarctation, hydroCephalus, Central bones short (rhizomelia), Crazy eyes (exophthalmos).
Lückenschädel Skull
Aka Lacunar skull
due to defective bone matrix
classically associated with Chiari II and neural tube defects
Wormian Bone Mnemonic
“Wormian PORK CHOPS”
Pyknodysostosis (sclerosing bone dysplasia; osteopetrosis with wormian bones; lysosomal storage disorder)
Osteogenesis Imperfecta (Collagen type I disorder)
Rickets (vitamin D deficiency [metephyseal fraying, splaying and cupping])
Kinky hair syndrome = Menke’s = X-linked recessive disorder AKA Fucked up Copper Metabolism -> fine, silvery brittle hair)
Cleidocranial dysostosis
Hypothyroidism/Hypophosphatasia
One too many 21 chromosomes (downs)
Primary Acro-osteolysis = Hajdu-cheney syndrome = connective tissue disorder
Acro-Osteolysis Mnemonic
the resorbed distal phalanx is “FO PINCHing”
F: familial, e.g. Hajdu-Cheney syndrome
O: other, e.g. polyvinyl chloride exposure, progeria
P: psoriasis/pyknodysostosis
I: injury, e.g. thermal burn, frost bite
N: neuropathy, e.g. diabetes mellitus, leprosy
C: collagen vascular disease, e.g. scleroderma, Raynaud disease
H: hyperparathyroidism
Pediatric scalp trauma - what are the three blood collections, superficial to deep, and which crosses sutures?
- Caput Succedaneum = Crosses sutures - resolves on its own
- Subgaleal hemorrhage = crosses sutures, potentially life-threatening and seen after vacuum extractions
- Cephalhematoma = limited by suture lines
scalp laceration + depressed fracture = ?
? + dural tear = ??
Compound fracture
compound fracture + dural tear = penetrating fx
What is a leptomeningeal cyst?
“Growing Skull Fracture”
fractured noggin + dural tail with leptomeninges herniating through torn dura and over time progressively widens
What is sinus pericranii
Dural venous sinus in the pericranium
CHARGE Syndrome
Coloboma
Heart Defects
Atresia (Choanal)
Retard
Genitourinary Abnormalities
Ear Anomalies
Treacher Collins syndrome
Mandibulo-facial dysostosis
problems with first and second branchela arches.
concave curve of the horizontal ramus
PHACES syndrome
P: posterior fossa malformations (e.g. Dandy-Walker malformation)
H: hemangiomas
A: arterial anomalies
C: coarctation of the aorta and cardiac anomalies
E: eye (ocular) anomalies
S: subglottic hemangiomas / sternal cleft / supraumbilical raphe
Neonatal CXR
High volumes + Perihilar Streaky
MNoPpT
Meconium aspiration (+pneumothorax)
Non-GB neonatal pneumonia (with pleuarl effusions)
TTN
Neonatal CXR
low volumes (or normal) + Granular
Surfactant deficiency disease
GBS neonatal pneumonia
Potter Sequence
Babies who can’t Pee in utero develop POTTER seuqence
Pulmonary hypoplasia
Oligohydramnios
Twisted face
Twisted skin
Extremity defects
Renal failure
Pleuropulmonary blastoma
Normal 2nd trimester US + infant with lung tumor = Primary lung tumor (rather than a congenital malformation)
Big Fucking Mass in the chest of a 1-2 yo.
Shouldn’t have eat up rib (Askin tumors often do)
Multilocular cystic nephroma 10% of the time.
Inflammatory Myofibroblastic Tumor (IMT)
Most common primary lung tumor in children. It is benign (unlike pleuropulmnonary blastoma). These are solid masses, lobulated and calcified, and T2 bright because of their myxoid appearance.
What is neonatal atypical peripheral atelectasis?
Uncle to round pneumonia in children - essentially the same thing but peripheral.
What is Swyer-James syndrome?
Post-infectious obliterative bronchiolitis (not hyperexpanded).
DDx mnemonic for GI obstruction in a child?
AAIIMM
Appendicitis, Adhesions
Inguinal hernia, Intussusception
Midgut volvulus, Meckels
Pentalogy of Cantrel
- Omphalocele
- Ectopia cordis (abnormal position of the heart)
- Diaphragmatic defect
- Pericardial defect or sternal cleft
- Cardiovascular malformations
Liver mass DDx ages 0-3
The 3 H’s
- Infantile Hemangioma (basically a hemangioma. . .)
- Hepatoblastoma (HCC of childhood, most common primary liver tumor < 5 yo, associated with Wilms, AFP and precocious puberty [due to bHCG secretion])
- Mesenchymal Hamartoma (cystic mass with large feeding portal vein branch)
Liver mass DDx > 5yo
HCC
Fibrolamellar HCC
Undifferentiated embryonal sarcoma = pissed off cousin of the mesenchymal hamartoma.
Caroli’s disease is associated with what renal stuff?
Polycystic disease and medullary sponge kidney
Vesicoureteric Reflux Grading
grade 1: reflux limited to the ureter
grade 2: reflux up to the renal pelvis
grade 3: mild dilatation of ureter and pelvicalyceal system
grade 4: tortuous ureter with moderate dilatation blunting of fornices but preserved papillary impressions
grade 5: tortuous ureter with severe dilatation of ureter and pelvicalyceal system loss of fornices and papillary impressions
What cancer does urachal remnants and bladder exstrophy get?
AdenoCA
DDx for neonatol renal lesions
Nephroblastomatosis (nephrogenic rests)
Mesoblastic nephroma (ie neonatal Wilms)
Multicystic dysplasic kidney
DDx for renal masses around age 4
how about teenager?
Wilms, Wilms variants (clear cell and rhabdoid), lymphoma, multilocular cystic nephroma
Teenager = RCC and Lymhoma
Syndrome associated with Wilm’s tumor
- Overgrowth
- Beckwith-Wiedemann syndrome = wilms, omphalocele, hepatoblastoma, macroglossia
- Soto = macrocephaly, retarded, ugly face
- Non-Overgrowth
- WAGR = wilms, aniridia, gential, growth retard
- Drash = Wilms + pseudohermaphroditism, progressive glomerulonephritis
What subtype of testicular cancer is associated with Peutz-Jeghers syndrome?
sertoli (non-germinomatous)
How does one tell between a dead thanatophoric dwarf from a dead Asphyxiating thoracic dystrophy (Jeune) dwarf?
By looking at their vertebral bodies - The Jeune Dwarf bodies will be normal.
Additional Randomw Dwarf Trivia
What are these dwarves:
Ellis-Van Crevald
Pseudoachondroplasia
Pyknodysostosis
- Ellis-Van Crevald = dwarf with polydactyly
- Pseudoachondroplasia = weird thing not present at birth, and spares the skull
- Pyknodysostosis = dwarf with osteopetrosis, wide angled jaw, wormian bones and acro-osteolysis.
Gorlin Syndrome
Bifid ribs, falx calcifications, basal cell cancers, odontogenic keratocysts (lytic jaw lesions).
Radial dysplasia ddx
VACTERL, Hold-Oram, Fanconi Anemia, Thrombocytopenia Absent Radius (TAR, which have a thamb).
talocalcaneal coalition signs
- Continuous C sign - produced from an absent middle facet
- talar beak
Calcaneonavicular coalition signs
Anteater sign = elongated anterior process of the calcaneus
Lucent Metaphysla Bands DDx
LINE
Leukemia
Infection (TORCH)
Neuroblastoma mets
Endrocrine (Rickets, Scurvy)
Currarino Triad
CurrArino TriAd
Anterior Sacral Meningocele
Anorectal Malformations
Sacrococcygeal Osseous Defect (scimitar sacrum).
Spinal Dysraphisms
Open
Closed (w/ and w/o subcutaneous mass)
- Open
- Myelomeningocele
- Myelocele
- Closed with a subcutaneous mass
- Meningocele
- Lipomyelocele/lipomyelomeningocele
- terminal myelocystocele
- Closed without subcutaneous mass
- Intradural lipomas
- fatty filum (fibrolipoma of the filum terminale)
- tight filum terminale
- Dermal sinus
Situs Solitus
Normal
Situs Inversus
Complete mirror image
situs ambiguous = heterotaxy, of which you can have right or left “isomerism”
Visceral heterotaxy with thoracic right isomerism
Everything on the right moves towards the left
- Two fissures in left (ie b/l minor fissures - minor fissure should only be on right and moves over to left)
- Asplenia (right liver moves into its place to the left)
- Increased cardiac malformations (think of the right middle lobe crossing over and fucking up the heart)
- Reversed aorta/IVC
situs ambiguous = heterotaxy, of which you can have right or left “isomerism”
Visceral heterotaxy with thoracic left isomerism
Everything on the left moves over to the right
- Absent minor fissure on the right (i.e. no minor fissures)
- Polysplenia (liver on left moves over to the right)
- Less cardiac malformations (just moves over from left to right)
- Azygous continuation of the IVC
Weigert Meyer Rule
Inferior pole (IR) refluxes; orthotopic ureteral insertion
Upper pole = ureterocele = obstructs (the more dialated one); ectopic ureteral insertion (inferior and medial)
Intralobar versus extralobar pulmonary sequestrations
intra = recurrent Infections, presents later
Extra = presents earlier due to “extra things” ie malformations
Anterior mediastinal mass
< 10 yo; > 10 yo
< 10 yo = Thymus
> 10 yo = lymphoma
Posterior mediastinal mass
< 10 yo
> 10 yo
< 10 yo think malignant (ie neuroblastoma)
> 10 year old think benign
BFM in the chest of a kid
Askin tumor = 10 + yo with eaten up rip
Pleuropulmonary blastoma age < 2 yo
Microcolon ddx
Meconium ileus = CF and the contrast fills TI
distal ileal atresia = the contrast won’t get to TI
Peds pancreatic mass at years 1, 6 and 15 yo
1 yo = pancreatoblastoma
6 yo = adenoCA
15 yo = SPEN
Double aortic arch
most common vascular ring
impression on anterior trachea and posterior esophagus
Right art with aberrant left subclavian
second most common vascular ring
anterior depression on trachea and posterior impression on esophagus
pulmonary sling
anomalous origin of the left PA from right PA, which courses between trachea and esophagus
posterior impression on trachea and anterior impression on esophagus
(only sling to produce ANTERIOR impression on the esophagus
Left aortic arch with aberrant right subclavian
not a real ring or sling
dysphagia lusoria
What cancers are associated with horseshoe kidneys?
Wilms, TCC and RENAL CARCINOID
