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310 Cards in this Set

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What is the Caudal Regression Syndrome?

abnormal fetal development of the lower spine which can lead to a variety of problems ranging from partial absence of the tail bone regions of the spine to more severe cases involving major malformation of the lower vertebrae, pelvis and spine. Minor cases may be more of less without symptom, whereas severe cases may come with major birth defects, neurological impairment and incontinence.
What is Sirenomelia?
rare congential deformity, legs are fused together, giving the appearance of a mermaid. This condition is found in approximately one out of every 70,000 live births[1] (about as rare as conjoined twins) and is usually fatal within a day or two of birth because of complications associated with abnormal kidney and bladder development and function. It results from a failure of normal vascular supply from the lower aorta in utero. Sirenomelia is associated with maternal diabetes
What are Tamm-Horsfall Proteinuria?
also known as stasis nephropathy
perinatal renal insult of less severe degree than acute cortical necrosis
prolonged nephograms on IV urograms,renal ultrasound:
increased echogenicity in central portions of pyramids, preservation of hypoechoic peripheral medulla
due to deposition of Tamm-Horsfall protein in the tubules,Benign course, usually resolves completely
What is Wilms Tumor?
nephroblastoma,
3rd most common kiddie tumor (most common abd tumor)
arises from embryonal renal tissue (nephroblastomatosis)
large
only 10% are calcified
4-10% bilateral
mets --> lungs, para-aortic nodes
associated with:
aniridia
Beckwith-Wiedemann syndrome
hemihypertrophy
Drash syndrome
What is Aniridia?
rare congenital condition characterized by the underdevelopment of the eye's iris. This usually occurs in both eyes. It is associated with poor development of the retina at the back of the eye resulting in visual loss.
What is Beckwith-Wiedemann syndrome?
big tongue, organomegaly (liver, kidneys, pancreas, heart)
omphalocele, umbilical hernia or diastasis recti
What is the Drash Syndrome?
male pseudohermaphroditism
progressive nephritis
What is Nephroblastomatosis?
persistent primitive renal tissue (normally present up to 36 weeks)
potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor
associated with:
hemihypertrophy
sporadic aniridia
pseudohermaphroditism
Klippel-Trenaunay syndrome
Beckwith-Wiedemann syndrome
splenic agenesis with hepatic malformation
findings:
kidneys may be enlarged
deformity of the pelvocalyceal system
US: subtle subcapsular nodules (hypo-/iso-/hyperechoic)
CT: hypodense subcapsular nodules after contrast enhancement
What is HSP?
acute, self-limited arteritis
often follows Strep infection
symptoms:
purpura
nephritis
abdominal pain
joint pain
thick SB folds
thumbprinting in SB or colon
What is CCAM?
Congenital cystic adenomatoid malformation is a congenital disorder similar to bronchopulmonary sequestration. In CCAM, usually an entire lobe of lung is replaced by a non-working cystic piece of abnormal lung tissue. This abnormal tissue will never function as normal lung tissue.
In most cases the outcome of a fetus with CCAM is very good. However in a rare few cases the cystic mass grows so large as to limit the growth of the surrounding lung and cause pressure against the heart. In these situations, the CCAM can be life-threatening for the fetus.
What is Klippel-Feil Syndrome and it's associations?
fused vertebrae
esp. cervical spine (C3-C4)
elevation of scapula (Sprengel deformity)
omocervical bones
GU abnormalities (66%)
renal agenesis (33%)
deafness (33%)
What is Sprengel Deformity?
congenital elevation of scapula
rotated --> inferior edge points medially,uni- or bilateral
almost always a/w Klippel-Feil syndrome, +/- omocervical (or "omovertebral") bones:
joins scapula to C5 or C6
What is the Omovertebral bone?
bony connection between the elevated scapula and one of the cervical vertebrae, usually C5 or C6
What is MURCS Syndrome?
Mullerian aplasia, renal aplasia and cervico-thoracic somite dysplasia
What is Mayer-Rokitansky-Kuster-Hauser syndrome or simply Rokitansky syndrome?
Agenesis of vagina and uterus is also called Mullerian aplasia
Transient Tachypnea of the Newborn
Transient tachypnea of the newborn (TTN) is a self-limited disease common in infants throughout the world and is encountered by all physicians who care for newborn infants. Infants with TTN present within the first few hours of life with tachypnea, increased oxygen requirement, and arterial blood gases that do not reflect carbon dioxide retention. When managing TTN, observing for signs of clinical deterioration that may suggest other diagnoses and for the development of respiratory fatigue is important
Meconium Aspiration
intra-uterine fetal distress ==> defecation of meconium

* term + post-term neonates
o NOT preemies
o NOT kids of diabetic moms

* diffuse HYPERaeration
o pulm interstitial emphysema (PIE)
o pnuemomediastinum + non-tension ptx in 33%
* patchy atelectasis + consolidation

* lung dz usually resolves spontaneously
* morbidity d/t intrauterine cerebral anoxia
Meconium Ileus
obstructed bowel due to impacted, tenacious meconium

* no air-fluid levels (probably d/t adherent bowel contents)

DDx:

* ileal atresia
* Hirschsprung dz
* anal atresia

a/w cystic fibrosis (CF)
Meconium Plug
functional colonic inertia"

* Dx/Tx: Gastrografin enema (DDx: Hirschsprung dz, meconium ileus)
* F/U with another enema for confirmation

* a/w maternal diabetes
copper beaten skull
c/w chronic hydrocephalus
elevated icp
What are germinal matrix hemorrhages associated with
<34 weeks
<1500g
Periventricular leukomalacia
Periventricular leukomalacia (PVL) is the most common ischemic brain injury in premature infants. The ischemia occurs in the border zone at the end of arterial vascular distributions. The ischemia of PVL occurs in the white matter adjacent to the lateral ventricles. The diagnostic hallmarks of PVL are periventricular echodensities or cysts detected by cranial ultrasonography. Diagnosing PVL is important because a significant percentage of surviving premature infants with PVL develop cerebral palsy (CP), intellectual impairment, or visual disturbances
rickets
relative or absolute deficiency of vitamin D

* presents by 3-6 months, almost always < 2 yrs

xray:

* loss of zone of provisional calcification
* wide physis (> 1 mm)(increased osteoid)
* cupping, fraying + irregularity of metaphyses
* bowing of long bones
* decreased bone density
* rachitic rosary
* periosteal reaction
Tibial Bowing
* anterior bowing
o OI
o hypophosphatasia
o thanatophoric dwarfism
o neurofibromatosis
o --> if fractured, doesn't heal normally

* posterior bowing
o often bilateral
o d/t fetal malposition
o fracture will heal normally
Osteogenesis Imperfecta
brittle bone disease": abnormalities of collagen/collagen production

Type I
autosomal dominant
age at presentation: 2-6 years
Type II (congenital lethal OI)
autosomal recessive
pre or perinatal death (pulmonary hypoplasia)
Type III (severe prograssive OI)
autosomal dominant
marked progressive limb and spine deformity
Type IV
autosomal dominant
most mild form

* demineralization, cortical thinning
* multiple fractures with pseudoarthrosis
* exuberant callus formation
* blue sclerae
* presenile deafness
* dentinogenisis imperfecta
* wide sutures + Wormian bones
Wormian Bones
small ossicles within cranial suture lines

* osteogenesis imperfecta
* cleidocranial dysostosis
* cretinism
* idiopathic

* Down syndrome
* hypophosphatasia
* Menkes kinky-hair syndrome
* progeria
* pyknodysostosis
Cleidocranial Dysostosis
disorder of ossification/development of midline bones

autosomal dominant, 33% sporadic

* skull
o cranial dysplasia
o Wormian bones
o basilar invagination

* clavicles
o aplasia / hypoplasia, usually lateral portion

* other skeletal abnormalities
o small, high scapula
o wide symphysis pubis
o acro-osteolysis
basilar invagination
* dens projects into base of skull

o McGregor's line > 4.5 mm
o Chamberlain's line > 3 mm

* may --> obstructive hydrocephalus

etiology:

* 1' anomalies of occiput, atlas, axis
* softened bone: rickets / osteomalacia, Paget's dz
* fibrous dysplasia
* dysostoses: OI, achondroplasia, cleidocranial dysostosis

Cf: platybasia
Aplasia of Distal Clavicle
absent distal clavicle

* cleidocranial dysostosis
* rheumatoid arthritis
* hyperparathyroidism
acro-osteolysis
loss of terminal tufts of digits

* scleroderma / connective-tissue disease
* psoriatic arthritis
* Reiter disease
* frostbite(thumbs spared)/burns

* leprosy
* polyvinylchloride exposure
* hyperparathyroidism
* Lesch-Nyhan syndrome (they bite 'em off)
* cleidocranial dysostosis
* progeria

* pyknodysostosis (aplasia/hypoplasia of distal digits)
* sarcoidosis
* epidermolysis bullosa
pyknodysostosis
* autosomal recessive
* dense, sclerotic bones
[Cf: osteopetrosis (sporadic, not inherited)]

Features:

* open cranial sutures + fontanelles
* Wormian bones
* dolichocephaly

* sclerotic vertebrae
* fractured long bones

* short, stubby hands
* partial agenesis/aplasia of terminal phalanges
[simulates acro-osteolysis]
toddler's fracture
non-displaced spiral fx of tibia

* no h/o trauma
* tender swelling of tibia
* +/- periosteal new bone

* main problem: make correct dx

DDx:

* infection
* tumor
* non-accidental trauma (abuse)
slipped capital femoral epiphysis (SCFE)
* boys > girls
* age 10 - 15 yrs
* blacks > whites (unlike CHD + Legg-Perthes disease)
* related to height, weight, athletic activity

* if BILATERAL ==> renal osteodystrophy

* femoral epiphysis slips POSTERIORLY and MEDIALLY
* use frog-leg + lateral projections

* complications:
o AVN
o acute cartilage necrosis (chondrolysis)
o varus deformity
is a salter 1
developmental dysplasia of the hip
aka: congenital hip dislocation (CDH or CHD)
* females (80-90%), 2/3 are firstborn
* white > blacks
* associated:
o breech delivery (6x cephalic),C-section, torticolis, family history

* due to ligamentous laxity from increased circulating maternal estrogen, not acetabular dysplasia
* unilateral LEFT (70%); bilateral in only 5%

* Putti's triad
1. supero-lateral migration of femoral head
2. increased acetabular angle
3. small capital femoral epiphysis
Salter-Harris fractures
classification of epiphyseal fractures
I
pure epiphyseal separation
if non-displaced, jt effusion may be only sign
II
metaphyseal fracture + epiphyseal separation
III
epiphyseal fracture
IV
vertically oriented fx thru epiphysis + metaphysis
V
crush injury of epiphysis (not detected acutely)

* most common: type II
* type I (best) --> type V (worst)
* seen in kiddies before the physes close
Tillaux fracture
fracture of LATERAL aspect of distal tibia

* analog of Salter III fx of distal tibia in kids:
o distal tibial epiphysis closes in 12-18 months
o MEDIAL portion closes first
Little Leaguer's elbow
avulsion fx of MEDIAL epicondyle
congenital infection: ToRCHS syndrome
T
Toxoplasma
R
Rubella
C
Cytomegalic inclusion disease (CID, CMV)
H
Herpes
S
Syphilis

* transplacentally acquired
* congenital infection

* celery-stalk metaphyses, esp. long bones
* intracranial calcification

* decreased growth
* vascular stenosis (aorta, pulm artery)
DDX Neonatal Respiratory distress
surfactant deficiency
TTN
Meconium Aspiration
Neonatal Pneumonia
intussusception
* 75% in kids <2 y/o
o ileocolic (75%), ileo-ileocolic (15%)
o 90% idiopathic
o leading point: lymphoid tissue (possibly increased 2' to enteritis)

* in older kids + adults (ileo- or colocolic)
o Meckel diverticulum
o Peyer's patches
o lymphoma
o large mesenteric nodes
o duplications
o polyps

* "currant jelly" stools
* "coiled spring" on BE
*
* ** transient intussusception: a/w sprue
Meckel diverticulum
* rule of 2's
o 2 cm wide
o 2 feet from I-C valve
o males 2:1
o 2% of population

* antimesenteric (unlike duplication cyst)
* +/- ectopic gastric/pancreatic tissue
Meckel scan
* agent: Tc-99m pertechnetate

* prep:
o pentagastrin -- increase acid production
o cimetidine -- block secretion of acid
o glucagon -- decrease bowel activity
what are the u/s findings in intussusception?
# Transverse sonography of the mass reveals a swirled pattern of alternating hyperechogenicity and hypoechogenicity representing the alternating layers of mucosa, muscularis, and serosa. target or donut sign.

Longitudinal sonography reveals a sandwichlike appearance of the alternating loops of bowel with a loop-within-loop appearance.
Rule of 3's with Intussusception
when reducing intussusception 3 ft high, 3 min per try, 3 tries
Henoch-Schoenlein syndrome
acute, self-limited arteritis
* often follows Strep infection

* symptoms:
o purpura
o nephritis
o abdominal pain
o joint pain

* thick SB folds
* thumbprinting in SB or colon
tracheo-esophageal fistula
Type Frequency Description
A 10% esoph atresia w/o fistula
B 1% esoph atresia w/ upper fistula
C 80% esoph atresia w/ LOWER fistula
D 2% "K" type: atresia w/ upper & lower fistulae
E 7% "H" type

VATER syndrome
VATER syndrome
V
vertebral anomalies
vascular anomalies

A
anal atresia

T
tracheo-esophageal fistula

E
esophageal atresia

R
renal anomalies
radial dysplasia
Klippel-Feil syndrome
fused vertebrae
esp. cervical spine (C3-C4)

elevation of scapula (Sprengel deformity)
omocervical bones

GU abnormalities (66%)
renal agenesis (33%)

deafness (33%)
cervical spine fusion (ankylosis)
posterior elements
JRA

vertebral bodies
block vertebrae
Klippel-Feil
Sprengel deformity
congenital elevation of scapula
rotated --> inferior edge points medially
uni- or bilateral

almost always a/w Klippel-Feil syndrome

+/- omocervical (or "omovertebral") bones:
joins scapula to C5 or C6
hemihypertrophy
unilateral enlargement (one digit to entire 1/2 of body)
associated with benign + malignant neoplasms:

kidney
nephromegaly
medullary sponge kidney
polycystic kidney disease
Wilms tumor / nephroblastomatosis
adrenal
cortical hyperplasia / adenoma / adenoCa
pheochromocytoma
liver
focal nodular hyperplasia
hepatoblastoma
hemangioendothelioma
gonads
phakomatoses
nephroblastomatosis
persistent primitive renal tissue (normally present up to 36 weeks)
potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor

associated with:
hemihypertrophy
sporadic aniridia
pseudohermaphroditism
Klippel-Trenaunay syndrome
Beckwith-Wiedemann syndrome
splenic agenesis with hepatic malformation
findings:
kidneys may be enlarged
deformity of the pelvocalyceal system
US: subtle subcapsular nodules (hypo-/iso-/hyperechoic)
CT: hypodense subcapsular nodules after contrast enhancement
Pulmonary lymphangiectasia
rare
dilated lymphatic channels secondary to either abnormal embryonic development of the lymphatic system or obstruction of lymphatic drainage. The dilated lymphatics cause a coarsely nodular or reticular pattern in the lungs, usually developing early in infancy
Pulmonary hemangiomatosis
rare
Recurrent hemorrhage into the lungs in patients with idiopathic pulmonary hemosiderosis eventually leads to chronic diffuse haziness or reticula in the lungs, representing pulmonary fibrosis
histiocytosis X
aka: Langerhans cell histiocytosis LCH

intense proliferation of reticulohistiocytic cells

* eosinophilic granuloma (EG)
o 60-80% of histiocytosis X
o age 5 - 10 yrs most common
o best prognosis

* Hand-Schuller-Christian disease
o age 1 - 3 yrs

* Letterer-Siwe disease
o age 0 - 1 yr
o worst prognosis
eosinophilic granuloma
* 60-80% of histiocytosis X
* age 5 - 10 yrs
* presentation: bone pain, local swelling, irritability

* bones
o 50 - 75% solitary / monostotic
o skull/mandible (50%): "punched-out" lucencies, "hole within a hole", "button sequestrum", "floating teeth"
o spine/pelvis (25%): vertebra plana (most common pediatric cause)
o long bones (15%): medullary lucency +/- thin sclerotic rim

* lungs
o involved in <10%, signals worse prognosis
o apical reticulonodular infiltrates
o honeycomb lung
Hand-Schueller-Christian disease
chronic diseminated form of histiocytosis X

* age 1 - 3 yrs most common
* skull and mandible lesions similar to EG, but more numerous
* "geographic skull", "floating teeth"

* adenopathy
* enlarged liver and spleen
* skin lesions
* diabetes insipidus
* exophthalmos
* lung disease
Letterer-Siwe disease
Acute diseminated form of histiocytosis X

* age 0 - 1 yr
* worst prognosis (70% mortality)

* visceral involvement: hepatosplenomegaly
* purpura, anemia, lymphadenopathy
* bone: extensive lytic skull lesions, "raindrop" skull
3 subtypes of Histiocytosis X
Hand-Schuller-Christian disease
Eosinophilic Granuloma
Letterer-Siwe disease
Causes of Miliary Nodules
Infection
Tuberculosis
Histoplasmosis
Viral
Idiopathic pulmonary hemosiderosis
Metastatic disease
hypoplastic left heart syndrome
underdevelopment of the left side of the heart characterized by:

* aortic valve atresia
* hypoplastic ascending aorta
* hypoplastic/atretic mitral valve
* endocardial fibroelastosis

most common cause of CHF in neonate

* 25% of cardiac deaths in 1st week of life
* prognosis if untreated: 100% fatal by 6 weeks

hemodynamics:

* pulmonary venous return is diverted from LA to RA through ASD
* RV supplies pulmonary artery, ductus arteriosus, descending aorta (antegrade flow), aortic arch, ascending aorta, coronary circulation (retrograde flow)
* leads to RV work overload and CHF

Rx:

* Norwood procedure (palliative) - three stage surgery that results in the RA feeding the pulmonary circulation and the RV feeding the systemic circulation.
* transplant
Congenital Pulmonary Hypoplasia
hypoplasia or absence of the ipsilateral pulmonary artery
a/w TOF, TA
tetralogy of Fallot
underdevelopment of pulmonary infundibulum

* 8% of all congenital heart disease
* most common CHD with cyanosis after 1 year of life

TETRAD:

1. obstruction of RV outflow tract; usually at the pulmonary infundibulum, occasionally at pulmonary valve
2. VSD
3. RV hypertrophy
4. aorta overriding the interventricular septum

associated with:

* bicuspid pulmonary valve (40%)
* stenosis of left pulmonary artery (40%)
* right-sided aortic arch (25%)
* TE fistula
* Down syndrome
* anomalies of coronary arteries (10%)
* forked ribs, scoliosis
pulmonary venolobar (scimitar) syndrome
* always involves aplasia of one or more lobes of right lung

* variably involves:
o partial anomalous pulmonary venous return (scimitar-shaped vein)
+ usually drains to IVC; but portal vein, hepatic vein, RA possible
o absent or small pulmonary artery perfusing abnormal lung segment
+ arterial supply from thoracic/abdominal aorta or celiac axis
o anomalies of hemidiaphragm on affected side
o anomalies of bony thorax or thoracic soft tissues
What causes pulmonary agenesis
insult during 4th week of fetal life
diastrophic dwarfism
short-limbed dwarfism ("diastrophic" means twisted, thus scoliosis)

x-ray features:

* short flared metaphyses with flattened epiphyses in tubular bones of extremities
* equinovarus deformity of feet -- major joints subluxed
* spine: kyphoscoliosis with gradual decrease in interpediculate distance toward caudal end of L-spine
* hands
o "hitch-hiker's thumb" - widely abducted
o hypoplastic first metacarpal
o other metacarpals too broad, esp. at growing end
o carpal bones with too many centers ossified
Ellis-van Creveld syndrome
chondro-ectodermal dysplasia

* hereditary (esp. seen in Amish of Pennsylvania)

* polydactyly (100%)
* congenital heart dz (60%)

* abnormalities of cutaneous appendages (skin, hair, nails)
Potter syndrome
I
autosomal recessive ("infantile") polycystic disease

* "microscopic" (1-mm) cysts
* US: +/- increased echo, no gross cysts

II
multicystic dysplastic kidney ("renal dysplasia")

* US: multiple cysts, usually unilateral

III
autosomal dominant ("adult") polycystic disease

* usually not present until adult life
* US: multiple cysts

renal agenesis

associated with:

* oligohydramnios
* abnormal facies
* hypoplastic lungs
prune belly (Eagle-Barrett) syndrome
* absent abdominal musculature
* undescended testes
* dilated ureters & calyces
* clubbed feet
* heart & lung abnormalities

* ** predominantly in males
Swyer-James syndrome
"unilateral hyperlucent lung", Macleod syndrome

* may be bilateral

* hyperlucency + air-trapping
* decreased pulmonary arteries
* paucity of bronchial subdivisions +/- proximal bronchiectasis

* h/o early + recurrent lower respiratory tract infections
* possibly 2' to obliterative bronchitis/bronchiolitis (adenovirus?)
Air trapping results in a lung that changes very little in size between inspiration and expiration. This important feature helps to distinguish the hypoplastic Swyer-James lung from the congenitally hypoplastic lung
cystic fibrosis
* probably autosomal recessive
* common in whites, 1:2000
* clinically: resp. disease, GI symptoms

complications:

* bronchiectasis
* pneumothorax
* hemoptysis
* lobar atelectasis
* pulmonary HTN
* cor pulmonale {normal-size heart may represent CMG}
* large, opacified sinuses

DDx: Riley-Day syndrome
Riley-Day syndrome
familial dysautonomia

* autosomal recessive
* seen in Jewish infants

* malfunction of autonomic nervous system
* possibly associated with catecholamine release + beta-NGF
* hypersecretion of mucous glands

* XR resembles cystic fibrosis
A right-sided aortic arch is the key radiographic clue to the presence of ?
an obstructing vascular ring
pulmonary sling
aberrant course of LEFT pulmonary artery between trachea + esophagus, compressing the trachea posteriorly

left pulmonary artery comes off the r pulm artery

* may cause obstructive emphysema of the right lung if the right main stem bronchus is compressed
Lateral radiographs demonstrate increased retrotracheal opacity, tracheal narrowing, and anterior tracheal bowing
Right ascending aortic arch, Aberrant left subclavian artery that passes posterior to the esophagus\
Ligamentum arteriosum or Persistent ductus arteriosus stretching from the left subclavian artery to the pulmonary artery anterior to the trachea
patent ductus arteriosus (PDA)
* 9% of all CHD; M:F = 1:2
* associated with: prematurity, birth asphyxia, rubella, coarctation, VSD, trisomy 18 + 21

* mostly assymptomatic
* CHF (rare) usually by 3 months in large L-R shunts (PDA increases shunt volume)

findings (mimicks VSD):

* LA enlargement
* enlarged PA
* increased pulmonary vasculature
* enlarged RV + LV
* "railroad track": calcified ductus arteriosus

PDA beneficial in:

* tetralogy of Fallot
* Eisenmenger pulmonary hypertension
* interrupted aortic arch (supply lower extremity via PDA)
congenital infections
ToRCHS syndrome

T
Toxoplasma
R
Rubella
C
Cytomegalic inclusion disease (CID, CMV)
H
Herpes
S
Syphilis

* transplacentally acquired
* congenital infection

* celery-stalk metaphyses, esp. long bones
* intracranial calcification

* decreased growth
* vascular stenosis (aorta, pulm artery)
aortic coarctation
* most frequent cause of PVH + CHF in 2nd + 3rd weeks of life

associated with

* bicuspid aortic valve (50-80%)
* calcific aortic stenosis (d/t bicuspid valve)
* PDA or VSD (in most)
rf:figure 3 sign
post rib notching T4-T8 (only occurs when child is 7 yoa
neonatal pulmonary venous hypertension
* with cardiomegaly
o hypoplastic left heart
o critical aortic stenosis
o cor triatriatum
o pulm venous atresia

* normal heart size
o infradiaphragmatic TAPVR
What is the best way to determine abnormal lung?
inspiratory and expiratory frontal views of the chest or fluoroscopy can be helpful. The lung that changes the least in volume between inspiration and expiration is the abnormal lung
congenital lobar emphysema
* caused by bronchial cartilage abnormality
* SOLID mass at birth: dilated alveoli filled w/ fetal lung fluid

* usually in UPPER lobes (incl. RML)
* Tx: surgical lobectomy

Cf: cystic adenomatoid malformatio
cystic adenomatoid malformation of lung
* only true pulmonary cystic dz of newborn
* three xray types:
1. multicystic, walls of varying thickness
2. multicystic, one large dominant thin-walled cyst
3. solid

* mediastinal shift common
* cysts often contain fetal lung fluid

* Tx: surgery
Causes of Unilateral Obstructive Emphysema
Bronchial foreign body
Mucous plug
Congenital lobar emphysema
Bronchial stenosis/atresia
Tuberculosis
Vascular anomalies
Mediastinal masses
congenital absence of pulmonary valve
* BIG central PAs
* big RV
congenital cardiomyopathy
* endocardial fibroelastosis
* myocarditis
* glycogen storage dz (Pompe's)

* anomalous origin of left coronary artery from pulmonary artery
endocardial fibroelastosis
* unknown etiology
* diffuse thickening of endocardium
* few live > 2 yrs

* limited to LEFT chambers in 80%

* causes restrictive cardiomyopathy
congenital heart disease
* INCREASED PULMONARY BLOOD FLOW
o Acyanotic
+ L -> R shunts
o Cyanotic
+ admixture ("T") lesions

* NORMAL / DECREASED PULMONARY BLOOD FLOW
o Acyanotic
+ normal
+ aortic coarctation
+ cor triatriatum
+ cardiomyopathy
+ congenital mitral stenosis
+ hypoplastic left heart
o Cyanotic
+ tetralogy of Fallot
+ pulmonary atresia
+ TAPVR III
+ Ebstein anomaly
total anomalous pulmonary venous return (TAPVR)
admixture lesion: cyanosis, increased pulmonary blood flow

* incidence 2%
* must have R->L shunt for survival (eg, ASD)
* Darling's classification
o Type I = supracardiac (55%) - "Snowman heart"
o Type II = cardiac (30%)
o Type III = infracardiac (12%)

associated with

* ASD (needed for survival)
* asplenia - 3/4 of cases have TAPVR

see congenital heart disease
admixture lesion
cyanosis + increased pulmonary blood flow

* transposition (TGV)
* truncus arteriosis
* TAPVR
* tricuspid atresia (sort of "intracardiac TAPVR")
* single ventricle
* common atrium
* hypoplastic left heart
* DORV (double outlet right ventricle)
asplenia / polysplenia syndromes
probably different degrees of the same disorder

* asplenia (Ivemark syndrome, bilateral right sidedness)
o more severe
o right lung lobar anatomy bilaterally, bilateral eparterial bronchi
o associated with cardiac, GI/GU and situs anomalies
+ TAPVR(~100%),ECD(85%)and many others
o Howell-Jolly bodies - RBC inclusions

* polysplenia (bilateral left-sidedness)
o left lung lobar anatomy bilaterally, bilateral hyparterial bronchi
o multiple spleens/splenules
o bilateral SVCs, azygos continuation of IVC
o associated with cardiac(less common than in asplenia), GI/GU and situs anomalies
hypoplastic left heart syndrome
underdevelopment of the left side of the heart characterized by:

* aortic valve atresia
* hypoplastic ascending aorta
* hypoplastic/atretic mitral valve
* endocardial fibroelastosis

most common cause of CHF in neonate

* 25% of cardiac deaths in 1st week of life
* prognosis if untreated: 100% fatal by 6 weeks

hemodynamics:

* pulmonary venous return is diverted from LA to RA through ASD
* RV supplies pulmonary artery, ductus arteriosus, descending aorta (antegrade flow), aortic arch, ascending aorta, coronary circulation (retrograde flow)
* leads to RV work overload and CHF

Rx:

* Norwood procedure (palliative) - three stage surgery that results in the RA feeding the pulmonary circulation and the RV feeding the systemic circulation.
* transplant
congenital insensitivity to pain
* autosomal recessive

* neuropathic joints
* micro- and macrofractures
* epiphyseal separation
* osteomyelitis (mandible, fingers, toes)

DDx:

* congenital insensitivity to pain with anhidrosis (aut. rec.)
* hereditary sensory radicular neuropathy (aut. rec.)
* congenital sensory neuropathy (aut. dom.)
* familial dysautonomia (aut. rec.)
* Lesch-Nyhan syndrome (X rec.)
congenital renal cysts
* congenital solitary cyst
* multilocular cyst

* multicystic disease (renal dysplasia)
* polycystic disease
o autosomal-recessive ("childhood") form
o autosomal-dominant ("adult") form

* medullary sponge kidney
* medulary cystic disease

see also: Potter syndrome
multicystic dysplastic kidney disease
* most severe form of renal dysplasia
* most common cause of palpaple abdominal mass in infants

* unilateral (90%; fatal if bilateral)
* ureter/renal artery absent or hypoplastic
* NOT associated with other cysts or with periportal fibrosis

* Potter type II
congenital renal osteodystrophy
tubular form of renal osteodystrophy

* vitamin D-resistant rickets
* Fanconi syndrome
* renal tubular acidosis
vitamin D-resistant rickets
* X-linked dominant defect in renal tubular resorption of phosphate
* presents in 1st yr
* progressive limb deformities

xray:

* less severe changes than other rickets
* presents later

DDx features:

* family hx
* normal serum calcium
* marked hypophosphatemia (decreased PO4)
* no secondary hyperPTH
congenital sinus tumor
* midline depression or tract
* lined with stratified squamous epithelium
* most common sites:
o lumbosacral area
o occiput

* epidermoid or dermoid cyst anywhere along tract
congenital syphilis
* Wimberger sign
* periostitis

* part of ToRCHS complex
congenitally short esophagus
very rare

findings:

* nonreducible intrathoracic gastric segment
* short straight esophagus
* circular narrowing at GE junction frequently with ulcer
* GE reflux

see also: hiatal hernia
Which organism is associated with pneumatocele formation
Staph
An important clue to the correct diagnosis of diaphragmatic hernia?
is the absence or paucity of gas-filled bowel loops within the abdomen
Congenital diaphragmatic hernias most often occur through?
foramen of Bochdalek, which lies posteriorly and medially in each hemidiaphragm
Where do the foramen of Morgagni and Bochdalek lie?
Morgagni: anterior
Bochdalek: Post/Medial
Which cells produce surfactant
Type 2 Alveolar
surfactant decreases surface tension of the alveoli
What causes BPD
positive pressure ventilation
high O2 concentration
which damages structually immature lung
low birth weight/prematurity are predisposing factors
What are the 2 phases of BPD
Edematous Phase
Bubbly Phase
Retained fetal lung fluid
result of delayed clearance of the fluid normally present in the fetal lung.
common in infants delivered by cesarean section, presumably caused by the lack of squeezing of the chest as it passes through the vaginal canal
Pulmonary lymphangiectasia
associated with congenital heart disease or generalized lymphangiectasia
ECMO is used for which conditions
congenital diaphragmatic hernia meconium aspiration syndrome neonatal sepsis
pneumonia
What are 2 clues to a subpulmonic effusion
Flattening of the diaphragm
Lateral displacement of the dome
Unilateral Pleural effusions likely represent?
pneumonia
Sources of Diffusely Hazy or Reticular Lungs in the Neonate
Decreased lung volumes
Poor inspiration
Hyaline membrane disease
Normal to increased lung volumes
Retained fluid
Aspiration (amniotic fluid/meconium)
Pneumonia
Pulmonary edema
Pulmonary lymphangiectasia
Possible Causes of Pleural Effusions
Unilateral

Pneumonia/empyema
Chylothorax
Iatrogenic
Trauma
Intra-abdominal inflammation
Intrathoracic neoplasm
Ruptured aneurysm of ductus arteriosus

Bilateral

Renal disease
Lymphoma
Neuroblastoma
Congestive heart failure
Collagen vascular diseases
Fluid overload
bronchogenic cyst
abnormality of primitive foregut (ventral segment = tracheobronchial tree; dorsal segment = esophagus)

* lined with columnar respiratory epithelium
* CT: water density (50%); higher density (50%); may have air/fluid level

# mediastinal brochogenic cyst (86%)

* associated with spinal abnormalities
* M:F = 1:1
* stridor, dysphagia; usually assymptomatic
* may communicate with tracheal lumen
* location: posterior mediastinum (50%), pericarinal (35%), superior mediastinum (14%); usually on right

# intrapulmonary bronchogenic cyst (14%)

* M>F; LL:UL = 2:1 (usually medial third)
* infection (75%), dyspnea, hemoptysis
* 36% will eventually contain air
broncho-pulmonary sequestration
* congenital lung malformation
* portion of lung receives blood supply from SYSTEMIC artery

* types
o intralobar
o extralobar

* usually LEFT lower lobe
intralobar sequestration
nonfunctioning portion of lung WITHIN visceral pleura of a pulmonary lobe

* LEFT lower lobe (67%); rarely in upper lobes

* arterial supply: SYSTEMIC, usually aorta
* venous drainage: usually via pulm. vv. (occ. systemic)

* usually does NOT communicate with bronchial tree
* cystic, may become infected

* not a/w other abnormalities

see: sequestration
Cf: extralobar sequestration
extralobar sequestration
"accessory lung": lung tissue in its own pleura

* LEFT lower lobe (90%)
* systemic arterial AND venous connection (unlike intralobar) results in L->R shunt

* a/w congenital anomalies of diaphragm:
o eventration
o paralysis
o ipsilateral hernia

* XR: homogeneous ST mass, rarely infected

see also: sequestration
Cf: intralobar sequestration
A mass connected to an unusually large vessel is likely
pulmonary arteriovenous malformation
central, oval-shaped nodule associated with overaeration of the involved lobe suggests
mucocele in a patient with bronchial atresia
Pulmonary hamartoma
benign congenital tumor flocculent calcifications
Ewing sarcoma
small, round-cell sarcoma of mesenchyme of medullary bone

* age 5 - 14 yrs
* most lethal of all 1' bone tumors

* any bone
o < 20 y/o: long bones
o > 20 y/o: flat bones (where there's still red marrow)

* purely lytic (62%), purely sclerotic (15%)
* periosteal reaction (onion-skin or perpendicular)

* similar lesion at different age
o < 5 y/o -- neuroblastoma
o > 30 y/o -- mets, reticulum cell sarcoma
DiGeorge syndrome
* absent thymus(aplasia) + parathyroids
o (branchial cleft anomaly)

* can't diagnose from CXR
o (stress or infection --> involution of thymus)
caused by faulty development of the third and fourth pharyngeal pouches
Cystic hygroma
congenital malformation of lymphatic origin that commonly arises in the neck. Cystic hygroma tends to be locally invasive and often extends into the mediastinum
dermoid / epidermoid tumor
"intracranial pearly tumor"

* congenital ectodermal tumor
* stratified squamous capsule secretes cholestrine + desquamated cells

* site:
o petrous apex / CPA (most common)
o suprasellar cistern (parasellar mass)
o cerebral hemisphere
o cerebellum / 4th ventricle

* CT:
o low density (d/t fat content); occ. high density
o no enhancement
o extra-axial
What causes GI duplication cysts
abnormal development of the posterior division of the primitive foregut.
they generally do not communicate with the esophagus
Aortic aneurysms in children can be seen in pts with
Trauma
Ehler-Danlos
Marfans
Ehlers-Danlos syndrome
* autosomal dominant
* connective-tissue disorder:
o hyperelasticity of skin, poor wound healing
o hyperextensibility of joints
o soft-tissue calcifications (spheroids)

* vascular lesions:
o aortic dissection
o aneurysms
o rarely: tortuous arch, ectatic pulmonary aa.
o tissue fragility ==> hematomas, aortic rupture after angio

* clinical types:
o gravis, mitis, benign hypermobile, ecchymotic, X-linked

* a/w ** medullary sponge kidney
Marfan syndrome
connective-tissue disorder: autosomal dominant, 15% sporadic

* cardiovascular system (prob. cause of death in 93%)
o aortic aneurysm (mostly ascending)
o dilatation of aortic sinuses
o aortic dissection
o coarctation

* musculoskeletal system
o tall stature, long limbs
o arachnodactyly
o lax joints

* spine
o scoliosis (60%) / kyphosis / pectus
o dural ectasia

* eye
o lens subluxation

Cf: homocystinuria
Coronary Artery Aneurysms are commomly seen in pts with
Polyarteritis Nodosa
Mucocutaneous Lymph node Syndrome
polyarteritis nodosa
necrotizing vasculitis of medium-sized arteries

* usually in male adults
* associated with hepatitis B antigen

* kidney: most frquently involved organ (85%)
o multiple small intrarenal aneurysms
o aneurysms may disappear (thrombosis) or appear in new locations
o arterial narrowing and thrombosis (chronic/healing stage)
o multiple small cortical infarcts
o associated with hypertension and renal failure

* chest involvement (70%)
o cardiomegaly/pericardial effusion (14%)
o wedge shaped/round peripheral infiltrates simulating PE (14%)
o interstitial lower lung field pneumonitis

* also may involve liver (66%), mesenteric vessels (50%), skeletal muscle (39%), skin (20%)
neuroblastoma
* most common solid abdominal mass of infancy (12% of all perinatal neoplasms)
* 3rd most common malignancy in infancy (after leukemia and CNS tumors)
* 2nd most common tumor in children (Wilms more common in older kids)

location: anywhere within sympathetic neural chain

* adrenal gland (36%): bilateral in 7-10%
* extraadrenal in sympathetic chain (18%)
* thorax and posterior mediastinum (14%)
* neck (5%); pelvis (5%)
* skull, olfactory bulb, cerebellum, cerebrum: (2%)
* other sites (10%); unknown primary (10%)

metastasis: bone (60%); regional nodes (42%); liver (15%); intracranial (14%); lung (10%)

see also: Hutchinson, Pepper, and blueberry muffin syndromes
Hutchinson syndrome
1. primary adrenal neuroblastoma
2. extensive skeletal metastases, particularly skull
3. proptosis (metastases to orbit)
4. bone pain
Pepper syndrome
* primary adrenal neuroblastoma
* massive hepatomegaly from metastases
blueberry muffin sign
* raised, purple skin lesions

* dermal metastases of neuroblastoma (stage IV-S)
Normal vascularity is seen in which cases of cong heart disease
uncomplicated valvular disease
coarctation of the aorta mild forms of cardiomyopathy
Asymmetry of pulmonary blood flow is most commonly seen in which cases of congenital heart disease
tetralogy of Fallot
persistent truncus arteriosus valvular pulmonic stenosis
An enlarged PA may represent?
generalized increased pulmonary blood flow poststenotic dilation caused by valvular pulmonic stenosis (pulmonary valve insufficiency caused by increased rv output


ventricular output
A small PA may represent?
Pulmonary outflow obstruction ie:
Truncus Arteriosis
Transposition of Great Vessels
A small Aorta may represent?
Hypoplastic Left heart
L to R shunts (ASD/VSD)
A large Aorta may represent?
(MC) post stenotic dilitation from aortic stenosis
Aortic Valve insufficiency
L to R shunting at great vessel level
PDA/PTA/TOF
Coarctation
A narrowing of the aorta where the aorta and pulmonary arteries are joined by the ductus arteriosus
PDA beneficial in:
* tetralogy of Fallot
* Eisenmenger pulmonary hypertension
* interrupted aortic arch (supply lower extremity via PDA)
Right Sided Aortic Arch
A/W
often just isolated anomaly
TA
TOF
right aortic arch
TYPES:

* with aberrant LEFT subclavian artery (5% ==> CHD)
o most common type of right arch
o second most common cause of vascular ring
* with mirror image branching (95% associated with congenital ht dz)
o 90% tetralogy of Fallot (TOF) (25%) ** decreased PBF
o 2.5% truncus (30-50%) ** increased PBF
o 1.5% transposition (TGV) (5%)

* with isolated left subclavian artery
Causes of Acyanotic HD with increased pulmonary vascularity
VSD
ASD
PDA
Eisenmenger syndrome
* pulmonary aterial HTN
o heart size normal (or slightly increased)
o big central PAs with abrupt tapering

* bidirectional or reversed cardiac shunt
Eisenmenger physiology
Reversal or balancing of an initial L to R shunt
atrial septal defect (ASD)
most common CHD presenting in adults

* 8-14% of CHD
* M:F 1:4
* secundum > sinus venosus > primum
* average lifespan: 40 yrs

CXR:

* shunt vascularity with >2:1 shunt
* normal aortic knob
* enlargment of pulmonary arteries
* loss of visualization of SVC due to RVH and clockwise rotation of heart

complications:

* atrial fibrillation
* pulmonary artery HTN (15%)
* Eisenmenger syndrome (6-9%, NOT related to ASD size)

Lutembacher syndrome: ASD + mitral stenosis
Lutembacher syndrome
atrial septal defect + mitral stenosis (ASD + MS)

* increased pulm blood flow
* cephalization (PVH)
Aortopulmonary window
failure of complete division of the primitive truncus arteriosus, which leaves a communication between the aorta and the PA just above the valves. similar to a PDA
transposition of the great vessels
"TGV" / transposition:
aorta arises from RV
pulm artery arises from LV
intracardiac shunt

increased pulmonary vascularity
cyanosis

xray:
heart size may be normal at birth
gradually enlarges: globular or egg-on-its-side appearance
cyanosis
A bluish cast to the skin resulting from increased deoxyhemoglobin in the blood.
Increased pulmonary blood flow and cyanosis = admixture lesions
TGV (transposition) -- most common cause on 1st day of life
TAPVR (total anomolous pulmonary venous return)
truncus arteriosis
tricuspid atresia with TGV (25% of TA cases)
single ventricle
common atrium
hypoplastic left heart
DORV (double outlet right ventricle)

Decreased pulmonary blood flow and cyanosis- right to left shunts
TOF (tetralogy of Fallot) -- most common overall
Ebstein's anomaly
tricuspid atresia without TGV (75% of TA cases)
pulmonic valvular atresia
truncus arteriosus
right aortic arch (30-50%)
high arch
elevaated left PA
VSD always immediately beneath common truncal valve
"waterfall" or "hilar comma" sign (types II + III)
Collet & Edwards classification

I
short main PA arises from left post-lat aspect
II
PAs arise separately from left post-lat aspect
III
each PA arises from lateral aspects
IV
PAs arise from dorsal aorta ("pseudotruncus")
probably tetralogy of Fallot with pulmonary atresia
truncus fails to divide into 2 separate structures the aort and the pulm artery
What is the most critical component of TOF
Pulmonary Stenosis and therefore degree of r to l shunting
Name the 4 components of TOF
VAPR
obstructed RV outflow tract
VSD
RVH
Aorta overriding septum
causes of right ventricular hypertrophy
pulmonary valve stenosis
pulmonary hypertension
tetralogy of Fallot
VSD
Causes of Right Aortic Arch
right aortic arch

--------------------------------------------------------------------------------
TYPES:

with aberrant LEFT subclavian artery (5% ==> CHD)
most common type of right arch
second most common cause of vascular ring
with mirror image branching (95% associated with congenital ht dz)
90% tetralogy of Fallot (TOF) (25%) ** decreased PBF
2.5% truncus (30-50%) ** increased PBF
1.5% transposition (TGV) (5%)

with isolated left subclavian artery
What drug is used to help maintain a PDA
Prostaglandin E1
Uhl disease
"parchment right ventricle"

focal or complete absence of RV myocardium
What changes on a cxr result from LVH
rounded appearance of left heart border
What changes on a cxr result from RVH
retrosternal fullness
upward displacement of cardiac apex
Williams elfin-facies syndrome
sporadic, congenital disorder

idiopathic hypercalcemia of infancy
supravalvular aortic stenosis
facial dysmorphism

associated with
medullary nephrocalcinosis
pancreatitis
What aare the 2 types of coarctation
preductal or infantile
juxtaductal or adult
Cor Triatriatum
pumonary venous obstruction secondary to an abnl connection of the pulm veins with the LA
Situs Solitus
Normal Position of the Viscera
Concordant
refers to structures that remain in normal relation to one another
Which skin lesion is associated with esophageal atresia?
Epidermolysis Bullosa
What are the 2 classications of gastric volvulus
organoaxial
mesoaxial
what are the dimensions of the sphincter found in pyloric stenosis
thickness > 3 mm
pyloric canal > 14 mm
duodenal atresia
double bubble sign
polyhydramnios

associated with
Down syndrome
VATER syndrome
NB: annular pancreas also may cause duodenal obstruction, and is associated with VATER syndrome
Ladd's Bands
Peritoneal bands in patients with malrotation
bands extend from cecum to porta hepatis
may cause duodenal obstruction
Apple peel small bowel
familial
diffuse atresia nd stenosis of small bowel
meconium ileus
obstructed bowel due to impacted, tenacious meconium

no air-fluid levels (probably d/t adherent bowel contents)

DDx:
ileal atresia
Hirschsprung dz
anal atresia
a/w cystic fibrosis (CF
meconium plug
"functional colonic inertia"

Dx/Tx: Gastrografin enema (DDx: Hirschsprung dz, meconium ileus)
F/U with another enema for confirmation

a/w maternal diabetes

caused by abnl peristalsis
meconium aspiration syndrome
intra-uterine fetal distress ==> defecation of meconium

term + post-term neonates
NOT preemies
NOT kids of diabetic moms

diffuse HYPERaeration
pulm interstitial emphysema (PIE)
pnuemomediastinum + non-tension ptx in 33%
patchy atelectasis + consolidation

lung dz usually resolves spontaneously
morbidity d/t intrauterine cerebral anoxia
What is the earliest manifestation of CF
Meconium Ileus
How 2 distinguish ileal atresia from meconium ileus?
ileal atresia has air/fluid levels.
tx=surgery
Hirschsprung disease
aganglionosis of myenteric plexus of colon
functional obstruction
aganglionic segment of normal caliber
continuous involvement, from rectum proximally

males (80%)
usually presents by 6 wks

complications:
enterocolitis
perforation

a/w Down syndrome

DDx:
ileal atresia
meconium ileus (no air-fluid levels)
dx made by biopsy
look for transition zone
Functional megacolon
spasm of the puborectalis muscle
hydrometrocolpos
accumulation of secretions in the vagina and uterus
dilatation of the vagina proximal to a congenital obstruction (e.g., imperforate hymen)
produces a palpable, fixed midline mass
may cause hydronephrosis

AXR: abdominal mass extending from pelvis
US: cystic mass posterior to bladder with scattered echoes due to cellular material and blood
Cf: hydrometros = uterine dilatation only (obstruction at cervix)
Cremin's M Line
Imperforate Anus

The M line is drawn horizontally through the junction of the middle and lower thirds of the ischium (M). The line demarcates the level of the puborectalis sling
What is a common cause of gastritis in infants that leads to vomiting and bleeding
Milk allergy
Where does regional enteritis commonly occur
terminal ileum
necrotizing enterocolitis
"NEC"

pneumatosis intestinalis
submucosal: initial finding, mult. cystic lucencies; looks like stool (!)
subserosal: linear lucencies
portal vein: +/- transient; not significant

etiology: preemie, perinatal stress, intestinal ischemia
survivors may develop intestinal strictures

caused by hypoxia/ischemia
pneumatosis intestinalis
multiple thin-walled, non-communicating, gas-filled cysts located in subserosa +/- submucosa with normal mucosa and muscularis

primary pneumatosis intestinalis (15%)

secondary pneumatosis intestinalis (85%)
ischemia
necrotizing enterocolitis
mesenteric vascular disease
trauma (sigmoidoscopy, biopsy, BE, postsurgical anastomosis)
infection (primary infection, parasites, perforated jejunal tic)
inflammation
inflammatory bowel disease
connective tissue disease (esp. scleroderma)
Whipple disease
pulmonary dz (COPD, artificial ventilation)
colonic obstruction (air dissecting distally)
What is the hallmark of NEC
pneumatosis intestinalis

always look for air in the portal vein
pneumatosis cystoides
"pneumatosis cystoides intestinalis"
multiple gas filled cysts of various sizes located in subserosa (sometimes in submucosa)

age: adulthood
location: predominantly in lower colon

X-ray:
best demonstrated on CT
segmental mucosal nodularity (DDx: polyposis)
complication: assymptomatic large pneumoperitoneum (may persist for months/years)
appendicitis
Alvarado score

M
migration of pain = 1 pt.
A
anorexia/acetone = 1 pt.
N
nausea/vomiting = 1 pt.
T
tenderness RLQ = 2 pts.
R
rebound = 1 pt.
E
elevated temp = 2 pts.
L
leukocytosis (>10K) = 1 pt.
S
shift to left (>75%) = 1 pt.

5-6 pts = compatible
6-7 pts = probable
8-9 pts = very probable
Omental infarction
mimics appendicitis clinically but can be distinguished from appendicitis on US and CT. The infracted omental segment has the appearance of a heterogeneous mass that is usually located between the anterior abdominal wall and the colon
Mesenteric adenitis
is a self-limiting inflammatory condition involving mesenteric lymph nodes and is frequently viral in etiology. US usually demonstrates a cluster of enlarged lymph nodes in the right lower quadrant and a normal appendix.
What group of children are predisposed to developing peritonitis
Nephrotic syndrome
Causes of cholecystitis in children
sickle cell disease, congenital obstructive anomalies of the biliary tract, total parenteral nutrition, furosemide treatment, dehydration, hemolytic anemia, and short gut syndrome
2 causes ofbiliary atresia/hepatitis in the neonatal period
hep b
CMV
alpha 1 antitrypsin def
Byler disease
alpha fetoprotein (aFP)
ELEVATED in:
wrong dates
multiple gestation
midline closure (neural-tube) defects
encephalocele
anencephaly
liver tumors
hepatoblastoma
hemangioendothelioma (occ., mild)
hepatocellular carcinoma (HCC, "hepatoma")

DECREASED in:
wrong dates
chromosome abnl (incl. Down syndrome)
pancreas divisum
unfused ducts of Wirsung and Santorini
incidence: 5-11%

21-45% get pancreatitis,
possibly due to stenosis of the minor ampulla
Meckel diverticulum
rule of 2's
2 cm wide
2 feet from I-C valve
males 2:1
2% of population

antimesenteric (unlike duplication cyst)
+/- ectopic gastric/pancreatic tissue
see: Meckel scan
What type of ectopic tissue may be found in a Meckel's diveticvulum
pancreas
gastric

25%
Abdominal calcifications in a neonate may be C/w
meconium peritonitis
Hutch bladder diverticulum
bladder tic near uretero-vesical junction
weakens detrusor muscle

may lead to VU reflux
How is VUR graded
Reflux into the distal ureter is grade I.
Reflux into the upper collecting system with no dilation of the upper tract is grade II.
Grade III reflux shows similar findings, but with mild blunting of the calices. All of these findings are exaggerated in grade IV reflux, with marked hydroureter and caliceal dilatation. When the ureter is massively dilated and tortuous and the upper tract is markedly dilated, grade V reflux is present
high-output hydronephrosis is rare but may be seen with
Bartter syndrome
diabetes insipidus
psychogenic water drinking
primary mega-ureter
aperistaltic segment of distal ureter
no anatomic obstruction

males (80%)
left > right
20% bilateral
diagnosis of exclusion

may be a/w UPJ obstruction, reflux
In ureteral duplication which ureter is in an ectopic location
superior pole
Ureterocele
saccular dilated segment of the distal ureter that invaginates into the bladder lumen and impedes the flow of urine from the ureter into the bladder
Causes of Renal Cysts
Single
Simple cyst
Caliceal diverticulum
Abscess
Multilocular cystic nephroma
Multiple
Multicystic dysplastic kidney
Polycystic kidney disease
Glomerulocystic disease
Medullary cystic disease (juvenile nephronophthisis)
Tuberous sclerosis
Turner syndrome
von Hippel-Lindau disease
Zellweger syndrome
Beckwith-Wiedemann syndrome
Meckel-Gruber syndrome
Causes of Echogenic Renal Pyramids (nephrocalcinosis)
Normal neonate
Tamm-Horsfall proteinuria
Sickle-cell disease
Hypercalciuria
Renal tubular acidosis
Medullary sponge kidney
Hyperparathyroidism
Drugs (furosemide, steroids, vitamin D)
Prolonged immobilization
Bartter syndrome
Williams syndrome
Autosomal recessive polycystic kidney disease
Storage diseases
Glycogen-storage disease type 1A
Hurler mucopolysaccharidosis
Lesch-Nyhan syndrome
Oxalosis
may be normal in newborns at the tips of the medullary pyramids
detrusor hyperreflexia
Upper motor neuron abnormalities, which occur above the level of the pons, result in loss of voiding control and spastic bladder
detrusor-sphincter dyssynergy
abnormal external sphincter contraction occurs during contraction of the detrusor muscle, preventing urination and increasing the pressure within the bladder. This condition, known as
Multiple bladder diverticula may be associated with which syndromes
Ehlers-Danlos syndrome, Williams syndrome,
Menkes syndrome
prune belly syndrome
Megacystitis may be seen in 2 syndromes
Prune Belly (Eagle-Barrett)
MMH Megacystis-microcolon-hypoperistalsis (MMH) syndrome
What is Megacystis-microcolon-hypoperistalsis (MMH) syndrome
disorder of smooth muscle in the urinary and GI tract. exclusively in girls Insufficiency of the abdominal musculature
bladder is very large and dysfunctional. Decreased intestinal peristalsis leads to poor evacuation of the colon in infants who survive. Bladder exstrophy occurs when the bladder is exposed through a large defect in the anterior abdominal wall. Marked widening of the symphysis pubis and splaying of the pelvic bones are noted on radiographs in these infants
rhabdomyosarcoma
extremely malignant tumor of totipotential mesenchyme
peak age: 3 - 5 yrs, 15 - 18 yrs

site:
pelvis / GU tract (39%)
head / neck (31%)

types:
embryonal (incl. sarcoma botryoides)
alveolar
pleomorphic

see also:
bladder rhabdo
sarcoma botryoides
bladder rhabdomyosarcoma
usually arises at trigone
causes outlet obstruction --> big bladder
DDx: hemorrhagic cystitis (small bladder, filling defects)
sarcoma botryoides
type of embryonal rhabdomyosarcoma
mass resembles "bunch of grapes"
originates submucosally, grows into lumen
What causes a hydrocele
Peritoneal fluid passes into the scrotum through a patent processus vaginalis.
most resolve by 2 yos
What types of Posterior urethral valves are there
1: Abnormal migration and insertion of the urethrovaginal folds result in sail-like flaps of tissue that arise at the base of the prostatic urethra below the verumontanum,
The valves cause obstruction to antegrade flow of urine, leading dilatation of the posterior urethra, bladder wall thickening and trabeculation, and VUR

2: consists of a nonobstructive mucosal fold rather than an obstructing membrane. 3: posterior urethral valves consist of a membrane caused by incomplete canalization in the region of the urogenital diaphragm

it is possible to have an anterior urethral valve but they are rare
Cryptorchidism
undescended testis
4% of term newborn boys
The majority of undescended testes will spontaneously descend into the scrotal sac by 1 year of age
Gonadoblastoma
neoplasm that occurs in children with intersex disorders, usually arising in the gonadal streaks or intra-abdominal testes of phenotypic females
nephroblastomatosis
persistent primitive renal tissue (normally present up to 36 weeks)
potential precursor to Wilms tumor: present in 30% of kidneys with single and 100% of kidneys with bilateral Wilms tumor

associated with:
hemihypertrophy
sporadic aniridia
pseudohermaphroditism
Klippel-Trenaunay syndrome
Beckwith-Wiedemann syndrome
splenic agenesis with hepatic malformation
findings:
kidneys may be enlarged
deformity of the pelvocalyceal system
US: subtle subcapsular nodules (hypo-/iso-/hyperechoic)
CT: hypodense subcapsular nodules after contrast enhancement

usually spont regress by 4 mos
Wilms tumor
nephroblastoma
3rd most common kiddie tumor (most common abd tumor)
arises from embryonal renal tissue (nephroblastomatosis)
large
only 10% are calcified
4-10% bilateral
mets --> lungs, para-aortic nodes

associated with:
aniridia
Beckwith-Wiedemann syndrome
hemihypertrophy
Drash syndrome

Cf: neuroblastoma (more likely calcified)
What should you always look for when ruling out torsion
spiral appearance of the spermatic cord
decreased flow
Causes of Renal Enlargement
Bilateral
Diffuse renal disease (e.g., nephrotic syndrome, glomerulonephritis)
Diabetic mother
Autosomal recessive or autosomal dominant polycystic kidney disease
Leukemia, lymphoma
Hemolytic uremic syndrome
Henoch-Schönlein purpura
Beckwith-Wiedemann syndrome
Glycogen-storage disease
Tuberous sclerosis
Nephroblastomatosis
Unilateral
Hydronephrosis
Duplication anomaly
Compensatory hypertrophy
Crossed fused ectopia
Multicystic dysplastic kidney
Renal abscess
Renal neoplasm
Renal vein thrombosis
What is the prognosis of Wlms tumor dependent upon
histology
resectability
It is important to look at what structures when considering a dx of Wilms tumor
extension into renal vein
IVC
RA
Claw sign
renal parenchymal stretched along the margin of a mass

MC= WIlms tumor
how does WIlms tumor usually present
assymptomatic abdominal mass
name 2 rare renal tumors
clear cell sarcoma
rhabdoid tumor
Similar radiographic entities when considering Wilms tumor
Renal Cell Carcinoma
Clear Cell Sarcoma (bone mets)
Rhabdoid Tumor (brain mets)
adrenal hemorrhage
neonate

* RIGHT more common, 10% bilateral
* birth trauma
* hypoxia
* septicemia
* congenital syphilis
* hemorrhagic disorders (hemophilia, etc.)

adult

* septicemia (Waterhouse-Friderichsen syndrome)
* tumor
* trauma

Notes:

* usually resolves in 4-6 wks
* adrenal insufficiency rare, even if bilat.
* may calcify
neuroblastoma
* most common solid abdominal mass of infancy (12% of all perinatal neoplasms)
* 3rd most common malignancy in infancy (after leukemia and CNS tumors)
* 2nd most common tumor in children (Wilms more common in older kids)

location: anywhere within sympathetic neural chain

* adrenal gland (36%): bilateral in 7-10%
* extraadrenal in sympathetic chain (18%)
* thorax and posterior mediastinum (14%)
* neck (5%); pelvis (5%)
* skull, olfactory bulb, cerebellum, cerebrum: (2%)
* other sites (10%); unknown primary (10%)

metastasis: bone (60%); regional nodes (42%); liver (15%); intracranial (14%); lung (10%)

contrast to Wilms it is poorly marginated and frequently extends into the chest

commonly calcifies, Wilms does not
Hutchinson syndrome
1. primary adrenal neuroblastoma
2. extensive skeletal metastases, particularly skull
3. proptosis (metastases to orbit)
4. bone pain
Pepper syndrome
* primary adrenal neuroblastoma
* massive hepatomegaly from metastases
blueberry muffin sign
* raised, purple skin lesions

* dermal metastases of neuroblastoma (stage IV-S)
Iodine-131-meta-iodobenzylguanidine
is a tracer that resembles norepinephrine and is metabolized by neuroblastoma, pheochromocytoma, and other catecholamine-producing tumors. Octreotide is a ligand for G-protein receptor cell membranes. These two tracers have improved detection of primary tumor and metastases in some cases
Adrenocortical carcinoma
highly malignant
CT and US characteristics similar to those of neuroblastoma
metastasizes to the lungs, liver, and regional lymph nodes.
The tumor frequently causes endocrine symptoms, such as virilization and Cushing syndrome. Hypercortisolism may cause an increase in retroperitoneal fat that is visible on CT and MR and is a clue to the diagnosis.
Adrenal elargement
adrenocortical hyperplasia which causes adrenogenital syndrome
cerebriform looking adrenals
infants treated with adrenocorticotropic hormone for spasm
Wolman disease
rare, familial xanthomatosis

* causes death early in infancy

* adrenal calcification
o diffuse, punctate calcifications throughout
o enlarged, normally shaped adrenals

* hepatosplenomegaly
Gallbladder Hydrops has been associated with what disease
Mucocutaneous lymph node syndrome
(Kawasaki's Disease)
choledochal cyst
* segmental dilatation of CBD

* F >> M
* 60% under 10 y/o

* classic clinical triad in < 40%
o jaundice, RUQ mass, abd pain
* choledochocele (type 3 cyst): bloops into duodenum

* 8% a/w Caroli disease
Caroli disease
communicating, cavernous ectasia of the intrahepatic bile ducts

* rare, autosomal recessive
* usually detected in childhood or early adulthood

* no cirrhosis or portal HTN

* predisposed to calculus formation
* recurrent cholangitis ==> liver abscesses ==> death
* increased risk of cholangiocarcinoma

* a/w medullary sponge kidney (renal tubular ectasia) in 80%
What usually causes choledocal cysts
anomalous junction of the common bile duct and pancreatic duct (abnormal pancreatobiliary junction [APBJ]). The APBJ allows pancreatic enzymes to reflux into the common bile duct, which may lead to inflammation and weakening of the bile duct wall
liver hemangioendothelioma
* females (67%)
* infants (90% before 6 m/o)

* sx: mass (hepatomegaly)
* cutaneous hemangiomas (45%)
* mildly increased aFP (occasionally)
* +/- CHF 2' to shunting (only 4%)

* usually diffuse, precludes surgery
* most involute spontaneously or with steroids

DDx:
o mesenchymal hamartoma
o cavernous hemangioma
o hepatoma
o hepatoblastoma (increased aFP)
o metastatic neuroblastoma (increased serum VMA)
Mesenchymal hamartoma
uncommon benign tumor seen most often in infants and young children
Hepatic adenomas are rare in childhood but have been reported in association with
Fanconi anemia
glycogen-storage disease type 1 Hurler disease
severe combined immunodeficiency.
hepatoblastoma
* 3rd most common intra-abd malignancy (after neuroblastoma + Wilms tumor)

* kids: 50% before 18 m/o, almost all < 3 y/o

* right lobe (75%), both lobes or multicentric (33%)
* mets at dx in 10% ==> local invasion, regional nodes, lungs

* increased aFP in 67-90%
* liver enzymes usually normal

associated with

* hemihypertrophy
* macroglossia
* sexual precocity

Assoc with Beckwith-Wiedemann and Familial adenomatous polposis
Beckwith-Wiedemann syndrome
* big tongue
* organomegaly (liver, kidneys, pancreas, heart)

* omphalocele, umbilical hernia or diastasis recti

a/w Wilms tumor

More info: Beckwith-Wiedemann syndrome [OMIM]
familial polyposis coli
* autosomal dominant (33% sporadic)

* adenomas ==> colon Ca
o absent at birth
o adenomas develop in teenagers

* associations:
o desmoid tumors
o SB adhesions

Cf: polyposis syndromes
splenomegaly
* infection
o SBE, TB, mono, brucellosis, syphilis, histoplasmosis, malaria, schistosomiasis
* sarcoidosis
* connective tissue diseases
o SLE, RA, Felty's
* lymphoma, leukemia, myelofibrosis, myeloid metaplasia
* anemias
* congestion
o portal HTN, cirrhosis, splenic vein thrombosis
* storage diseases
o Gaucher, Niemann-Pick, amyloidosis
* masses
o cyst, abscess, cavernous hemangioma
Cystic lymphangiomatosis
is a benign lymphatic malformation with a characteristic multiloculated cystic appearance
Hemophagocytic lymphohistiocytosis (HLH)
rare disease that consists of overactive histocytes and macrophages that phagocytize the normal cellular structures of the blood.
not truly malignant and is probably caused by an inappropriate immune reaction. HLH usually occurs in infants under 1 year of age and is characterized by hepatosplenomegaly, ascites , gallbladder wall thickening, lymphadenopathy, and pleural effusion.
Causes of Ascites
Newborn
Hydrops fetalis
Chylous ascites
Urinary tract obstruction
Iatrogenic (line perforation)
Intestinal perforation (necrotizing enterocolitis)
Older infants and children
Liver disease
Nephrotic syndrome
Portal vein obstruction
Traumatic intestinal injury
Peritonitis
Hypoproteinemia
Pancreatitis
Ruptured abdominal cyst
Intestinal lymphangiectasia
GI ischemia
Bile duct perforation

Cysts that contain gastric mucosa are detectable by scintigraphy using

technetium-99m-pertechnate
True congenital cysts of the pancreas are rare and occur chiefly in association with
autosomal dominant polycystic kidney disease
von Hippel-Lindau syndrome.
von Hippel-Lindau syndrome
retinocerebellar angiomatosis

* phakomatosis
* autosomal dominant (variable penetrance)

* hemangioblastoma: most frequent cause of death
o cerebellar (most common)
o also medullary and spinal
* retinal angiomatosis (45%)
* renal cell Ca: 2nd most common cause of death
* pheochromocytoma (17%)

* cortical renal cysts (75%)
* cysts in virtually any organ
* renal/liver hemangioma/adenoma
* pancreatic cystic neoplasms, islet-cell tumors
* paraganglioma
CSF pseudocyst
is a complication of the use of a ventriculoperitoneal shunt for the treatment of hydrocephalus
Solid-cystic papillary tumor of the pancreas
uncommon tumor that contains variable amounts of cystic and solid tissue
Tumors of the mesentery and omentum are primarily
Burkitt lymphoma
metastases
sacral agenesis
# agenesis or hypoplasia of sacrum

* part of caudal-regression syndrome

* a/w maternal diabetes
* +/- inherited

* possible features:
o absent lower extremities
o bladder/bowel impairment
chordoma
tumor of notochord remnants

* site:
o sacrum (45%)
o clivus (39%)
o spine

* destruction, no sclerosis
* +/- calcification (30-80%)

midline tumor

see also: para-sellar mass
Anterior sacral meningoceles
develop when a portion of the thecal sac protrudes anteriorly into the presacral space through a sacral defect
Currarino triad
partial sacral agenesis
anorectal stenosis
presacral mass
juvenile rheumatoid arthritis (JRA
* 15% seropositive
o F > M
o onset > 10 yrs
* 85% seronegative (Still disease)

* sites: KNEES, ANKLES, hands, wrists, feet

findings:

* decreased bone density
* early epiphyseal closure --> short bones
* ankylosis of spinal apophyseal jts

* big "squared" epiphyses
* wide intercondylar notch
* tibio-talar slant
Still disease
seronegative juvenile rheumatoid arthritis

* 85% of kids with RA
* uncommon: adult-onset Still dz

findings:

* fever
* rash
* adenopathy
* splenomegaly
cervical spine fusion (ankylosis)
* posterior elements
o JRA

* vertebral bodies
o block vertebrae
o Klippel-Feil
tibio-talar slant
* juvenile rheumatoid arthritis
* sickle-cell disease
* hemophilia
MURCS association
(müllerian duct, renal, and cervical vertebral defects)

Duncan described the MURCS association in 1979. It consists of a nonrandom association of müllerian duct aplasia, renal aplasia, and cervicothoracic somite dysplasia. The incidence of cervicothoracic vertebral defects, especially from C5-T1, is 80%. Other abnormalities may include Sprengel deformity, upper limb defects, and moderately frequent rib anomalies
rhabdomyosarcoma
* extremely malignant tumor of totipotential mesenchyme
* peak age: 3 - 5 yrs, 15 - 18 yrs

* site:
o pelvis / GU tract (39%)
o head / neck (31%)

* types:
o embryonal (incl. sarcoma botryoides)
o alveolar
o pleomorphic

see also:
bladder rhabdo
sarcoma botryoides
bladder rhabdomyosarcoma
* usually arises at trigone
* causes outlet obstruction --> big bladder
o DDx: hemorrhagic cystitis (small bladder, filling defects)
cardiac rhabdomyoma
associated with tuberous sclerosis
Tydings-McDuffie Act 1934
Bill to provide independence to the Philippines by 1946. A transition government with consitutional safeguards for the people would be in place in the meantime.
most common cause of adrenal mass in the neonate
Adrenal hemorrhage
what is th etriad that is pathognomonic for adrenal hemorrhage in the neonate
triad of jaundice, anemia, and an abdominal mass in a neonate is practically pathognomonic
Predisposing factors for the development of large ovarian cysts are
maternal diabetes, toxemia of pregnancy, and Rh incompatibility.
Differential diagnosis for cystic abdominal mass which persists after catheterization should include
enteric duplication cyst, choledochal cyst, mesenteric cysts, neonatal cyst, and hydrocolpos.
The differential diagnosis of bilateral hydroureteronephrosis should include
bladder outlet obstruction (secondary to posterior urethral valves, urethral polyp, tumor, ureterocele), ureteral atony secondary to infection, bilateral vesicoureteral reflux, bilateral ureteral ectopia (either from single systems or from upper poles of duplex kidneys), prune-belly, neuropathic bladder, and obstructive megaureter.
differential possibilities for diffuse bladder wall thickening,
one should mainly consider conditions of bladder outlet obstruction or neurogenic bladder dysfunction. Focal bladder wall thickening may be caused by previous surgery, e.g., ureteral reimplantation, and tumors, such as rhabdomyosarcoma, neurofibroma, or pheochromocytoma. Chemical cystitis, e.g., secondary to cyclophosphamide therapy, can cause mucosal edema mimicking any of the above conditions.
most common malignancy in childhood.
Leukemia
differential diagnosis of hypoechoic lesions of the testicles includes
germ cell tumors; however, given the clinical history, leukemia would be the most likely possibility.
DDX solid polypoid masses or mass-like lesions that may occur at the base of the bladder in a child include
focal cystitis, rhabdomyosarcoma, pheochromocytoma. Neurofibromatosis
Hamartomatous Polyp
differential diagnosis of an anterior urethral diverticulum is
Cowper's duct syrinx, which is typically located closer to the bulbar portion of the anterior urethra at the insertion of the Cowper's duct.
The differential diagnosis of a periumbilical infection should also include
persistent omphalomesenteric duct and simple omphalitis, urachal abnormality.
most common type of soft-tissue sarcoma in childhood.
Rhabdomyosarcoma
DDX Bladder Masses
hemangiomas and neurofibromas occur rarely in the bladders of children. It is also important to realize that acute viral cystitis in the child can occasionally appear as a bladder mass and can mimic bladder rhabdomyosarcoma.
differential diagnoses, in this case, in which there is a cystic mass behind the bladder in association with ipsilateral absence of the right kidney in the right renal fossa, include
ureterocele (with renal dysplasia), ectopic multicystic dysplastic kidney, and utricular (mullerian duct) cyst. Renal dysplasia and ectopic kidney
most common presacral mass in a child
Sacrococcygeal teratoma
differential diagnosis of a presacral mass in the newborn and child includes
sacrococcygeal teratoma, meningocele, lipoma, rectal duplication, abscess, and pelvic neuroblastoma
differential diagnosis of a complex suprarenal mass in the neonate is
adrenal hemorrhage, neuroblastoma, and (exophytic) hepatoblastoma. These entities can look similar on cross-sectional imaging. Laboratory value assessment and follow-up imaging may be needed to differentiate NBI from adrenal hemorrhage. In the older child, the differential diagnosis may also include Wilms' tumor. On cross-sectional studies, Wilms' tumor should be recognized as arising from the kidney with a tendency to displace vessels, while NBI not only displaces but also encases them.
most frequent cause of hydronephrosis in childhood, accounting for approximately 50% of cases,
Ureteropelvic junction (UPJ) obstruction followed in frequency by ureterovesical junction obstruction, ureteroceles, ectopic ureters, posterior urethral valves, prune-belly syndrome, and neuromuscular vesical dysfunction
most frequent renal anomaly
Ureteral duplication
DDX Multilocular Cystic Nephroma
Cystic wilm's
Cystic RCC
most frequent causes of renal parenchymal disease in childhood and adolescence are
acute pyelonephritis, glomerulonephritis, nephrotic syndrome, and hemolytic uremic syndrome. Less frequent etiologies include sickle cell anemia, glycogen storage disease, polycystic disease, lymphoma, polycythemia, and renal dysplasia.
differential diagnosis of Wilm's includes
multilocular cystic nephroma, mesoblastic nephroma, and renal cell carcinoma, lymphoma, clear cell, rhabdoid.
primary differential diagnoses of nephroblastomatosis are
lymphoma and leukemia
DDX Perinephric masses
be seen in retroperitoneal fibrosis (rare in children) and in renal lymphangiomatosis which produces fluid-filled rather than solid lesions.
DDX Wilm's
Clear cell sarcoma, Wilms' tumor, mesoblastic nephroma, and renal cell carcinoma can have appearances similar to that of malignant rhabdoid tumor. The presence of subcapsular or perinephric fluid collections should suggest either malignant rhabdoid tumor or clear cell sarcoma, since similar fluid collections have not been reported in the other tumors.
DDX Atypical Teratoma
other cystic ovarian lesions, such as simple cysts, hemorrhagic cysts, and cystadenoma.
DDX Vaginal obstruction in the neonate
In the neonate, obstruction may be the result of vaginal atresia or stenosis, a transverse septum, or a cloacal malformation
differential diagnostic considerations of scrotal swelling in the neonatal period include
meconium peritonitis or intraperitoneal blood tracking through the patent process vaginalis, hernia, and testicular tumor, especially teratoma
In prepubertal boys, the most common malignant tumor of the testes
yolk sac carcinomas (mean patient age, 2 years);
In prepubertal boys, the most common benign tumor of the testes
teratoma
In neonates and infants, causes of nephrocalcinosis include
furosemide therapy, renal tubular acidosis, ACTH treatment, and Williams' syndrome (idiopathic infantile hypercalcemia)
In older children and adolescents, the most common causes of nephrocalcinosis are
renal tubular acidosis and oxaluria. Less frequent causes include hypercalcemic states such as hyperparathyroidism, sarcoidosis, hypervitaminosis D, milk-alkali syndrome, malignancy, Cushing syndrome and hyperthyroidism; parenchymal renal diseases including chronic glomerulonephritis, tuberculosis, mycoses, and medullary sponge kidney; and vascular conditions such as acute cortical or tubular necrosis.
differential diagnosis of hyperechogenic medullary pyramids includes
precipitation of Tamm Horsfall proteins, vascular congestion, papillary necrosis, infection of the renal medulla with cytomegalovirus or Candida albicans, medullary fibrosis, and autosomal recessive polycystic disease.
DDX low T2 signal seminal vessicles
low T2 signal intensity material is secondary to intracellular methemoglobin from recent biopsy, prior radiation, amyloid, or hormone therapy
optimal location for an UVC tip
The optimal location for an UVC tip is in the inferior vena cava (IVC), or at the junction of the IVC and the right atrium and is defined by the catheter tip being located between the eighth and ninth thoracic vertebrae on plain radiography. The majority of UVC tips sited below this level are found to be in vessels in the liver proximal to the ductus venosus.
DDX infant presenting with a heterogeneous liver mass on ultrasound i
hepatic hemangioma
mesenchymal hamartoma
hepatoblastoma.
most common cause for a pancreatic mass in the pediatric population is
lymphoma
DDX Double bubble
Duodenal atresia
Duodenal web
Malrotation with midgut volvulus
Annular pancreas
Malrotation with crossing Ladd bands
Normal neonate imaged too early for distal progression of gas
Most common cause of macrocephaly in infants?

Benign enlargement of the subarachnoid spaces in infancy (BESSI)

What is the clinical triad of Prune Belly Syndrome?

Prune belly syndrome classically presents with a clinical triad of:


absent/hypoplastic abdominal wall musculature (causing bulging flanks and the wrinkled “prune” appearance of the abdomen), large trabeculated urinary bladder with hydroureteronephrosis and vesicoureteral reflux, and undescended testes (in males).

* Include prune belly syndrome in the differential diagnosis of a male neonate with radiographic signs of urinary bladder outlet obstruction.
* Rare in Females
* aka Eagle-Barrett syndrome