Pediatric radiology review/differentials

Lung Hypoplasia
Abn Muscle/Bone—Dysplasia, Syndrome (JEUNE)
Nervous System—Tri 21, Paralysis
BELL-SHAPED THORAX
Hemangioma
Tracheitis (membranous croup)
Retropharyngeal abscess
Epiglotitis
Croup
UPPER AIRWAY
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RAP’N FOREGUT
Round pneumonia
Abscess
Pseudotumor
Neoplasm (RARE) hamartoma, blastoma
Foregut malformations
PULMONARY MASS
Congenital lobar emphysema
CCAM
Sequestration
Bronchogenic cyst
BRONCHOPULMONARY FOREGUT MALFORMATIONS
O RENAL Mets
Osteosarcoma
Rhabdomyosacroma
Ewings/E. Gran
Neuroblastoma
Askin tumor/PNET
Lymphoma/Leukemia
Mets
CHEST WALL MASS (bill)
HPS
Spasm
Antral we
STOMACH DZ
Annular pancreas
Hematoma
Preportal Duodenum
Duodenal Stenosis
DUODENUM DZ
Duodenum
Annular pancreas
Volvulus
Ladds bands
DOUBLE BUBBLE
AA II MM
Adhesion
Appendicitis
Inguinal Hernia
Intussusception
Malortation with volvulus
Meckels/misc
SMALL BOWEL OBSTRUCTION
EVALUATE LEVEL OF DISEASE FROM LEVEL OF OBSTRUCTION:
RECTUMSIGMOIDLEFT COLONRIGHT COLONTERMINAL ILEUM
Microcolon secondary to proximal atresia
Hirschprungs (rectum)
Meconium plug/Small left colon syndrome (left colon)
Colonic atresia (entire colon)
Ileal Atresia (entire colon)
Meconium ileus
Jejunal atresia
MgSO4
Infant of a diabetic mother
MICROCOLON
UPJ
Reflux
Posterior urethral valves
Ectopic ureterocele
Prune belly
Primary megaureter
MCDK
HYDRONEPHROSIS
MCDK
Juvenile nephronopthesis
APCKD
ARPCKD
Glomerulocystic disease
Obstructive lesions
NUCS CAN DIFFERENTIATE FCN/Non FCN
RENAL CYSTIC DISEASE
Wilms—(chest mets) (>1 yr)
Rhabdoid—(brain mets) (1 yr)
Clear cell sarcoma—(bone mets) (1 yr)
Mesoblastic nephroma
Multilocular cystic nephroma
RCC (>3 yr)
Renal medullary carcinoma (sickle cell disease)
Pyelonephritis
RENAL MASS
Nephroblastomatosis
Pyelonephritis
Lymphoma/mets
Infarcts
Cysts
BILATERAL RENAL MASSES
Bilateral hydronephrosis
Glomerulonephritis
ARPCKD
ADPCKD
Bilateral renal vein thrombosis
Nephroblastomatsois
Beckwith Weidemann
BILATERAL ENLARGED KIDNEYS
NAP
Neuroblastoma
Adrenal hemorrhage/Adrenal cortical carcinoma
Pheochromocytoma
ADRENAL MASS
FUR
Fibroepithelial polyp
Ureterocele
Rhabdomyosarcoma
BLADDER MASS
Peritoneal
Meconium peritonitis (most common)

Renal
Wilm’s tumor
Nephrocalcinosis
Renal cysts
Urinary calculi
Ossifying renal tumor of infancy

Adrenal
Neuroblastoma
Hemorrhage
Wolman’s disease

Bladder
Hemorrhagic cystitis (Cytoxan therapy)

Gallbladder
Cholelithiasis

Bowel
Appendicolith
Meckel’s diverticulum fecalith

Liver
Hepatoblastoma
Granuloma

Spleen
Granuloma

ABDOMINAL CALCIFICATION
NEWBORN
Infantile hemangioma (solid)
Hepatoblastoma (solid)
Embryonal cell sarcoma (mixed)
1 YR
Mesenchymal hamartoma (cystic)
>3 YR
Hepatocellular carcinoma (variable)
LIVER MASS (newborn,1yr,3yr)
ECHO™
Enteric duplication
Choledochal cyst/mesenteric cyst
Hydrocolpos
Ovarian cyst
Teratoma
Meconium pseudocyst
CYSTIC ABDOMINAL MASS
SHOT
Scurvy
Hypophosphatasia
OI
Trauma
MULTIPLE FRACTURES
SCALPR
Scurvy/infection
Caffey
Accidental trauma
Leukemia
PGE2
Rickets
DIFFUSE PERIOSTEAL REACTION
Downs
JRA
Morquio
Trauma
ATLANTOAXIAL WIDENING
MODIC
Morquio
Osteogenesis imperfecta
Dwarf (thanatophoric)
Cushing’s syndrome
PLATYSPONDYLY
SALMON
Spinal cord tumor
AchondropLasia
Mucopolysaccharidosis
Osteogenesis imperfecta
Neurofibromatosis
POST VB SCALLOPING
Premature closure of sagittal suture (long skull)
SCAPHOCEPHALY = DOLICOCEPHALY
Premature closure of coronal/lambdoid sutures (short tall skull)
BRACHYCEPHALY = TURRICEPHALY
Unilateral early fusion of coronal/lambdoidal suture (lopsided skull)
PLAGIOCEPHALY
Premature closure of metopic suture (forward pointing skull)
TRIGONOCEPHALY
Premature closure of coronal, sagittal, lambdoid sutures
OXYCEPHALY
Intrauterine premature closure of sagittal, coronal, lambdoid sutures
CLOVERLEAF SKULL = KLEEBLATTSCHÄDEL
FONAR
Fibrous dysplasia
Osteogenesis imperfecta
Neurofibromatosis
Achrondroplasia
Rickets
TIBIAL BOWING
KIDS WITH SACRAL MASSES RANT
Rectal duplication cyst
Anterior meningocele
Neuroblastoma
Teratoma
SACRAL MASS
Trauma
JRA
Hemophilia
TB/infection
Trevor’s disease
KNEE POSSIBLE CASES
Septic effusion
Toxic synovitis
Legg Calve Perthes (school age)
Congenital dysplasia hip (neonate/infant)
Slipped capital femoral epiphysis (adolescent)
HIP POSSIBLE CASES:
TWILL
Trauma
Warfarin
Infection
Legg Calve Perthes
Leg dysplasia
FRAGMENTED EPIPHYSIS
TAR (thrombocytopenia – absent radius syndrome)
Holt Oram syndrome (cardiac, chest wall anomalies)
Fanconi’s anemia
Poland syndrome
VACTERL
RADIAL RAY (5)
Familial
Trisomy 13
Lawrence-Moon-Bardet-Biedel
POLYDACTYLY
Renal (55%)
Hydronephrosis
Multicystic dysplastic kidney
Mesoblastic nephroma
Renal vein thrombosis
Polycystic kidney disease

Gastrointestinal (15%)
Duplication cyst
Meconium pseudocyst
Mesenteric, omental cyst

Genital (15%)
Ovarian cyst
Hydrometrocolpos

Nonrenal retroperitoneal (10%)
Adrenal hemorrhage
Neuroblastoma

Hepatobiliary (5%)
Metastases
Hemangioendothelioma
Hepatoblastoma
Mesenchymal hamartoma
Choledochal cyst

ABDOMINAL MASS, NEONATE (BILL)
Renal (55%)
Wilms’ tumor
Hydronephrosis
Cystic disease
Hematoma
Multilocular cystic nephroma
Other tumors

Nonrenal retroperitoneal (25%)
Neuroblastoma
Rhabdomyosarcoma
Other tumors

Gastrointestinal (15%)
Appendiceal abscess
Intussusception
Neoplasm

Genital (5%)
Ovarian cyst
Hydrometrocolpos

Hepatobiliary (5%)
Hepatoblastoma

ABDOMINAL MASS, OLDER INFANT AND CHILDREN (BILL)
Extremities
Rhizomelic limb shortening
Trident hand: space between middle fingers in infancy
Metaphyseal flaring
Short, stubby fingers
Fibula longer than tibia

Skull
Craniofacial disproportion
Enlarged skull: normal-sized in reality but disproportionately large relative to body (intact membranous ossification)
Frontal bossing
Small skull base (endochondral ossification)
Small foramen magnum and jugular foramen: brainstem compression, jugular vein stenoocclusive disease
Hydrocephalus secondary to venous and CSF outflow obstruction at foramen magnum, jugular foramen
J-shaped sella
Foreshortened temporal bones
Short clivus
Small sphenoid bone
Prominent mandible
Platybasia

Spine
Short vertebral bodies with decreased AP diameter
Tall disk spaces
Decreasing interpediculate distance more inferiorly (opposite of normal)
Concavity of posterior vertebral bodies
Short pedicles: prone to spinal stenosis
Kyphosis is lower (at TL junction instead of thorax)
Exaggerated lumbar lordosis

Pelvis
“Tombstone” or “ping-pong paddle” iliac bones: short with squared iliac wings
Horizontal acetabular roof: decreased acetabular angle
Small sacroiliac notch

Thorax
Short ribs

Achondroplasia (BILL)
Occurs in neonates secondary to trauma, stress
Right (70%), left (20%), bilateral (10%)
Need to follow to resolution (neuroblastoma may hemorrhage)

Predisposing conditions
Traumatic delivery
Hypoxia
Sepsis
Maternal diabetes mellitus
Dehydration

Clinical
Hypovolemic shock
Anemia
Jaundice

Distinguish from neuroblastoma
Neuroblastoma is echogenic with diffuse vascularity
Adrenal hemorrhage is anechoic with no vascularity
Neuroblastoma is T2-hyperintense
Adrenal hemorrhage is T2-hypointense

Other findings
Initiallly appears as solid mass, increased density by CT early
Liquefaction occurs within 10 days to produce cystlike appearance
Wall calcification
Decreases with time

Adrenal hemorrhage, neonatal (BILL)
Nasal cavity, nasopharynx
Antrochoanal polyp
Meningo-encephalocele
Angiofibroma
Lymphadenopathy
Neuroblastoma
Rhabdomyosarcoma
Hemangioma
Lymphangioma

Oropharynx
Lymphadenopathy
Ectopic thyroid tissue
Hemangioma
Lymphangioma

Hypopharynx, larynx, trachea
Retention cyst
Papilloma
Hemangioma
Lymphangioma

Airway mass (BILL)
Nose
Pyriform aperture stenosis
Choanal atresia
Polyps
Tumor
Trauma
Encephalocele
Dacrocystocele

Nasopharynx
Adenoids
Lymphoma
Abscess
Angiofibroma
Teratoma
Rhabdomyosarcoma

Oropharynx
Palatine tonsils
Peritonsillar abscess
Macroglossia
Micrognathia
Teratoma
Lingual thyroid
Thyroglossal duct cyst
Ranula
Retropharyngeal cellulitis, abscess

Supraglottis
Epiglottitis
Aryepiglottic fold cyst
Hemangioma, lymphangioma

Glottis
Glottic stenosis
Cord paralysis
Papilloma
Trauma

Subglottis
Croup
Bacterial tracheitis
Stenosis
Granulomatous disease
Papilloma
Hemangioma, lymphangioma
Trauma

Trachea
Tracheomalacia
Innominate artery impression
Pulmonary sling (complete cartilaginous rings)
Vascular ring
Enlarged ascending aorta or pulmonary arteries
Foreign body (tracheal, esophageal)
Lymphoma
Granulomatous disease
Papilloma

Airway obstruction (BILL)
Ring of pancreatic tissue encircling duodenum at or above ampulla of Vater
Ventral pancreas does not completely rotate to left of duodenum; forms ring
May be symptomatic or asymptomatic
Clinical: pain, early satiety, vomiting
Increased incidence of PUD, pancreatitis
Usually diagnosed in infancy, childhood
May cause periduodenal fibrosis or stricture

UGI
Eccentric or concentric narrowing of descending duodenum
Mucosal effacement
Dilatation of proximal duodenum
May get reversal of peristalsis and dilatation of duodenum distal to narrowing

Treatment
Transection not recommended: may lead to duodenal narrowing, pancreatitis, pancreatic fistula
May need gastroenterostomy (fibrosis not relieved with pancreatectomy alone; need bypass)

Annular pancreas (BILL)
Failure of descent and separation of hindgut and genitourinary tract in 2nd trimester

MRI
Determine location of rectal pouch with respect to levator
Distinguish supralevator from infralevator types
Detect associated spinal anomalies

Types
Line drawn from coccyx to pubis
High (supralevator) malformation
Low (infralevator) malformation

High (supralevator) malformation
Rectum ends above levator sling
High association with GU (50%) and cardiac anomalies
Male: fistula to urethra or bladder
Female: rectovaginal fistula, hydrometrocolpos

Low (infralevator) malformation
Rectum ends below levator sling
Low association with GU anomalies (25%)
Male: anoperineal fistula
Female: fistula to lower portion of urethra, vagina, perineum

Anorectal anomalies, background (BILL)
VACTERL (90%)
Vertebral anomalies
Anorectal anomalies
Cardiac anomalies
Tracheoesophageal fistula
Renal anomalies
Limb anomalies

Caudal regression syndrome
Sacral anomalies (30%): tethered cord
Currarino’s triad
Anorectal anomaly
Sacral bone abnormality
Presacral mass

Genitourinary anomalies (30%)

GI anomalies
Duodenal atresia
Esophageal atresia

General
Oligohydramnios
Polyhydramnios

Maternal diabetes

Anorectal anomalies, associations (BILL)
Likely autosomal dominant
Acrocephalosyndactyly
Advanced paternal age may be associated
Mutation in fibroblast growth factor receptor 2 on chromosome 10

Features
Facial
Craniosynostosis: brachycephaly, turricephaly, cloverleaf skull
High prominent forehead
Hypoplastic sinuses
Flat posterior skull
Midfacial deficiency
Hypertelorism
Low-set ears
Prognathism

Limb
Syndactyly
Absent middle phalanges
Thumb and great toe are broad and malformed

Apert syndrome
Age > 4 years
Most common cause of small bowel obstruction
Variable presentation (atypical in 30-45%, WBC not elevated in 10%)

Plain film
Appendicolith (5-10%)
Air-fluid levels in RLQ
Sentinel loop
Mass in RLQ with displaced bowel loops
Obliterated properitoneal fat stripe
SBO
Free air is rare in perforation

US
More useful in thin patients and girls (useful to distinguish from gynecologic masses)
High frequency transducer with graded compression
Shadowing, echogenic appendicolith
Noncompressible, blind-ending tubular structure
> 6 mm diameter
RLQ fluid, phlegmon, abscess
Thickened appendiceal wall

Barium enema (rarely done)
Completely filled appendix excludes diagnosis
15% of normal appendices do not fill with contrast
Beak of barium at base of appendix (mucosal edema)
Irregularity of barium near tip of cecum
Deformity of cecum (abscess, mass effect)

CT
Appendicolith
Distended appendix
Periappendiceal soft tissue stranding
Wall thickening of cecum, terminal ileum
Perforation: SBO, inflammatory collections in RLQ and pelvic cul de sac

Appendicitis, backgroung (bill)
Proximal jejunal atresia
Absence of distal SMA
Shortening of small bowel distal to atresia
Absence of dorsal mesentery
Distal small intestine spirals around its vascular supply resembling an apple peel
Short intestine is prone to necrotizing enterocolitis
High mortality
Apple peel small bowel
Dystrophic calcifications of major arteries in infants
Death usually in first year of life secondary to coronary artery disease
Calcifications also in periarticular regions, ear lobes, kidneys
Spontaneous remission may occur

Treatment
Steroids
Estrogen
Disphosphonates

Arterial calcification of infancy
Urinary tract obstruction
Chylous ascites
Bile duct perforation
Neonatal liver disease
Ruptured ovarian cyst
Hemoperitoneum from ruptured liver, spleen
Meconium peritonitis
Syphilis
Ascites, neonatal (bill)
Bronchial foreign body
Swyer-James
Pulmonary hypoplasia
Pulmonary embolism
Asymmetric lucent lung
Omphalocele

Macroglossia

Gigantism

Visceromegaly
Pancreatic hyperplasia with hypoglycemia
Hepatomegaly
Renal enlargement

Tumors
Wilms’ tumor
Hepatoblastoma

Beckwith-Wiedemann syndrome (bill)
Pathology
Focal or total absence of extrahepatic ducts
Unknown etiology (severe hepatitis, sclerosing cholangitis, vascular component)
Periportal fibrosis
Intrahepatic small bile duct proliferation
Cirrhosis develops unless surgically corrected
Exclude cystic fibrosis: inspissated bile may cause similar findings

Demographics
Females > males

Associations
Trisomy 18
Abdominal heterotaxia syndromes (polysplenia)

Types
Correctable: Kasai procedure (portoenterostomy)
Noncorrectable

US
Initial imaging
Exclude choledochal cyst or other cause of obstruction
Absent gallbladder suggests biliary atresia (20% will have small or normal gallbladder)
Normal or enlarged GB suggests neonatal hepatitis (also presents with conjugated hyperbilirubinemia)
Hepatic parenchyma, intrahepatic ducts look normal

Cholescintigraphy
Tc 99m iminodiacetate (IDA) derivatives
One of most reliable ways to differentiate neonatal hepatitis from biliary atresia
Pretreatment with phenobarbital (5 mg/kg/day x 5 days): improves sensitivity
Adequate uptake and clearance by hepatocytes
Tracer never reaches GI tract (even on 24 hour delayed imaging)

Kasai procedure
Portoenterostomy
Complicated by ascending cholangitis and portal hypertension
Liver transplantation usually necessary by 3 years

Biliary atresia, background (bill)
Defect in lower abdominal wall, pubic area, anterior wall of urinary bladder, and dorsal aspect of urethra
Defect causes bladder to be open and mucosa to be continuous with skin
Most common congenital bladder abnormality
Overall rate: 1:50,000
Always associated with epispadia (male: urethra ends on dorsal aspect of penis; female: cleft of entire dorsal urethra)
Simple epispadia without exstrophy uncommon

Findings
Diastasis of symphysis (width correlates with severity of exstrophy)
Omphalocele confluent with exstrophic bladder
Cryptorchidism
Inguinal hernia
Acquired ureterovesical junction obstruction in untreated cases
After surgical repair: small bladder, reflux
Associated anomalies
Rectal prolapse
Bifid, unicornuate uterus
Spinal anomalies

Bladder exstrophy-epispadia complex (bill)
(congenital tibia vara)
Abnormal endochondral ossification caused by stress and compression
Deformity of medial proximal tibial metaphysis and epiphysis

Forms
Early onset (infantile)
Age: 1-3 years
Common
Bilateral (80%)
Low recurrence after surgery
Progressive deformity
Late onset (juvenile, adolescent)
Age: > 4 years
Uncommon
Unilateral (90%)
High recurrence after surgery
Less deformity

Both forms more common in obese black children
Clinical: bowed legs, pain
Severe cases may require tibial osteotomy

Findings
Fragmentation of medial tibial epiphysis
Irregular medial physeal line
Beaking of proximal medial tibial metaphysis
Bone bridge across medial physis while lateral growth plate remains open
Metaphyseal-diaphyseal angle (MDA) > 11 degrees
Tibiofemoral angle (TFA) > 15 degrees: tibia vara (alone not diagnostic for Blount’s disease)
Tibial bowing

Blount’s disease (bill)
Systemic diseases (most common)
Hypothyroidism (cretinism; typical hypoplastic T12 and L1)
Cyanotic congenital heart failure
Chronic pulmonary disease

Growth hormone deficiency (pituitary dwarfism)
Isolated GH deficiency
Craniopharyngioma
Infection

Peripheral tissue nonresponsiveness to growth hormone
African pygmies
Turner’s syndrome
Congenital short stature

Bone age, delayed
Aeration
Stomach: 2 hours after birth
Small bowel: 6 hours after birth
Rectum: 24 hours after birth

Pattern
Monotonous polygons of gas
Diameter less than distance between pedicles of T12
Small and large bowel cannot be differentiated

Bowel gas pattern, normal
Clinical
Poor feeding
Vomiting
Abdominal distention
Failure to pass or delayed passage of meconium

High (above distal jejunum)
Gastric atresia or web
Pyloric stenosis
Duodenal atresia, stenosis, or web
Duodenal duplication cyst
Malrotation, midgut volvulus
Jejunal atresia or stenosis

Low (below distal jejunum)
Ileal atresia or stenosis
Meconium ileus
Colonic atresia
Functional immaturity of colon
Small left colon
Meconium plug syndrome
Hirschprung disease
Imperforate anus

Bowel obstruction, neonate (bill)
Chronic lung disease of prematurity
Caused by oxygen toxicity and barotrauma of mechanical ventilation
Premature infants requiring prolonged ventilatory support

Stages
< 4 days Mucosal necrosis Findings similar to HMD 1 week Necrosis, edema, exudate Hazy density throughout both lungs 2 weeks Bronchial metaplasia Coarse lung markings Bubble-like lucencies Asymmetric aeration 1 month Fibrosis Coarse lung markings Bubble-like lucencies Asymmetric aeration Prognosis of stage 4 Mortality (40%) Minor handicaps (30%) Abnormal pulmonary function tests later in life Clinically normal by 3 years (30%) Patients with normal pulmonary function may only have exercise intolerance, predisposition to infection, and asthma Wilson-Mikity syndrome Bronchopulmonary dysplasia in absence of mechanical ventilation Controversial

Bronchopulmonary dysplasia (BPD) (bill)
Infantile cortical hyperostosis
Associated soft tissue swelling
Irritability, fever
1st few months of life
Unknown etiology
Self-limited and benign

Bones affected
Mandible
Clavicles
Ribs
Scapula
Humerus, ulna, radius
Femur, tibia

Findings
Periosteal bone formation
Sclerosis
Adjacent soft tissue swelling

Caffey’s disease
Metabolic
Healing stage of renal osteodystrophy
Hypoparathyroidism

Anemias (compensatory hematopoiesis)
Sickle cell disease
Thalassemia

Tumor
Leukemia
Lymphoma

Chronic decreased intracranial pressure
Shunts (most common cause of calvarial thickening)

Dilantin therapy

Fibrous dysplasia

Skeletal dysplasia
Pyle’s disease (craniometaphyseal dysplasia)
Camaruti-Engelmann’s disease (craniodiaphyseal dysplasia)

Calvarial thickening
Extremities
Micromelia
Bowing of long bones (campto = bent)

Skull
Enlarged skull
Abnormal mandible

Spine
Ossification defects
Scoliosis
Kyphosis in C spine

Pelvis
Tall, narrow iliac wings
Increased acetabular angle

Thorax
Absent scapulae
Defective tracheobronchial cartilage

GU
Sex reversal in males

Campomelic dysplasia
Bartonella henselae
Spread through contact with infected cat
Cat bite or scratch, contact with cat saliva on broken skin or conjunctiva of eye

Clinical
Papule or pustule at site of injury
Usually first sign
Local lymphadenopathy
Begins 2-3 weeks after exposure
May persist for months
Nodes may fistulize to skin and drain
Systemic symptoms: fever, fatigue, malaise, headache, anorexia, weight loss, splenomegaly

Diagnosis
Positive Bartonella serology
Bartonella henselae IFA test
Lymph node biopsy
Positive CSD skin test

Treatment
Heals spontaneously in immunocompetent patients
Antibiotics for immunosuppressed

Complications
Parinaud’s syndrome: eye infection with lymphadenopathy
Encephalopathy
Neuroretinitis
Osteomyelitis

Cat scratch disease
Rubella
CMV
Syphilis
Celery stalk appearance
Hemolysis
Sickle cell anemia (small spleen)
Thalassemia (large spleen)
Hereditary spherocytosis

Cystic fibrosis

Drugs
Furosemide

Hyperparathyroidism

Hyaline membrane disease

Cholelithiasis
Two forms
Conradi-Hunermann syndrome
X-linked
Normal intelligence
Flat nose
Asymmetric limb shortening
Cataracts (17%)
Chondrodysplasia punctata congenita
Autosomal recessive
Lethal
Rhizomelic form
Severe psychomotor retardation
Cataracts (72%)

Findings
Extremities
Rhizomelia
Stippled epiphyses
Spine
Coronal vertebral body clefts
Atlantoaxial instability
Spinal stenosis
Airway
Laryngeal, tracheal calcifications
Tracheal stenosis

Chondrodysplasia punctata
Inherited immunodeficiency
Phagocyte oxidase deficiency
Normal phagocytosis but impaired killing
X-linked or autosomal recessive

Clinical
Increased susceptibility to infections (fungal and bacterial infections)

Findings
Chest
Chronic or recurrent pneumonia
Bronchiectasis
Lung abscess
Mediastinitis
Neck
Suppurative cervical adenitis

Bones
Multifocal low-grade osteomyelitis
Little bone reaction for extent of infection
Abdominal
Hepatosplenomegaly
Gastric antral narrowing
Granulomas
CNS
Brain abscesses

Chronic granulomatous disease (CGD) (bill)
6% of all pediatric renal tumors

Demographics
Age: 1-4 years (peak age: 2 years)
M > F

Natural history and prognosis
Highly malignant with worse prognosis than Wilms’ tumor
High propensity for skeletal metastases
Other metastastic sites: lymph nodes, brain, liver, lungs
Long-term survival 60-70%

Imaging
Cannot be distinguished from Wilms’ tumor by imaging
Well-circumscribed solid renal mass without intravascular extension

Clear cell sarcoma (bill)
(talipes equinovarus)
Common (1:1000)
Bilateral in 50%
Left > right when unilateral
Boys > girls (3:1)
Normal talocalcaneal angle is 40 degrees on AP and lateral views (long axes of talus and calcaneus)
AP and lateral talocalcaneal angles are decreased in clubfoot

Features
Hindfoot varus
Distal bone (calcaneus) is medial to proximal bone (talus)
Seen on AP view
Decreased AP talocalcaneal angle (normal is 20-40 degrees)

Hindfoot equinus
Fixed plantar flexion of calcaneus (distal end points down)
Opposite would be hindfoot calcaneus (fixed dorsiflexion with distal end pointing up)
Seen on lateral view
Lateral talocalcaneal angle < 35 degrees Long axes of talus and calcaneus may appear to be parallel Forefoot varus and adduction (metatarsus adductus) 1st metatarsal is medial to line drawn through long axis of talus on AP view Base of 4th metatarsal medial to AP calcaneal axis Talonavicular subluxation Medial subluxation of navicular bone Infrequently diagnosed because ossification not yet occurred at time of clubfoot diagnosis

Clubfoot, background (bill)
Chromosomal
Trisomy 13, 18
Even if prenatal US fails to detect other malfomations, risk remains 6% for karytypic abnormality

Facial
Cleft lip and palate
Micrognathia

Cardiac

Limb
Hip dislocation
Contractures

CNS
Myelomeningocele
Hydrocephalus

Placental
Amniotic band syndrome

Syndromic
Gordon’s syndrome (camptodactyly and cleft palate; distal arthrogryposis type 3)
Distal arthrogryposis (fixated hands and feet)
Nail-patella syndrome
Muscular dystrophies
Pierre Robin syndrome

General
Oligohydramnios

Clubfoot, association (bill)
Progressive overdistention of alveoli in one or more pulmonary lobes (usually not entire lung)
10% have congenital heart disease (PDA, VSD)
Most present in neonatal period with respiratory distress
Treatment: lobectomy

Causes
Idiopathic (50%)
Airway obstruction with valve mechanism (50%)
Bronchial cartilage deficiency or immaturity
Mucus
Web, stenosis
Extrinsic compression

Lobar predilection
LUL (43%)
RML (32%)
RLL (20%)
Two lobes (5%)

CXR
Hyperlucent, hyperexpanded lobe
Initial radiographs may show soft tissue density related to retained fetal pulmonary fluid (density resolves and is replaced by progressive hyperlucency)
Shifted thymus

CT
Air trapped within alveoli: interstitial septae, bronchovascular bundles at periphery (not center) of lucency
Airspaces larger than those in adjacent normal lung
Pulmonary vessels attenuated

Congenital lobar emphysema (bill)
Acute laryngotracheobronchitis
Most commonly caused by parainfluenze virus
Age 6 months-3 years (younger age group than epiglottitis)
Most common cause of acute upper airway obstruction in young children

Clinical
Barking cough
Intermittent inspiratory stridor
After or during other symptoms of lower respiratory tract infection

Findings
Key view: AP view
Steeple sign: lose normal lateral convexities (shoulders) of subglottic trachea
Looks like Chrysler building (normal looks like Empire State Building)
Subglottic trachea becomes long, thin
Narrowing extends below level of pyriform sinuses
Lateral view: narrowing and loss of definition of subglottic tracheal lumen; hypopharyngeal overdistention
Epiglottis and aryepiglottic folds are normal

Croup (bill)
Abnormal development and configuration of acetabulum (increased acetabular angle and shallow acetabular fossa)
Increased ligamentous laxity around hip (effect of estrogen)
Much more common in females (9:1)
Increased incidence in whites, breech, oligohydramnios
70% on left; bilateral in 5%
If not detected and treated, may lead to chronic abnormalities of hip
Familial tendency: may be related to inherited abnormality in estrogen metabolism

Clinical
Neonatal screening
Asymmetric gluteal folds
Limited abduction of flexed hip
Shortening of one leg
Waddling gait
Positive click on Ortolani (relocation) or Barlow (dislocation) maneuvers

Treatment
Pavlik harness
Cast: flexion + abduction + external rotation
Salter osteotomy if chronic

Developmental dysplasia of hip, background (bill)
Putti’s triad
Superolateral displacement of proximal femur
Increased acetabular angle
Small capital femoral epiphysis

Hilgenreiner (Y-Y) line
Line drawn through both triradiate cartilages
Touches inferomedial aspect of each acetabulum
Forms acetabular angle with line drawn along acetabular roof

Acetabular angle
Angle between Hilgenreiner line and acetabular roof
Normally < 30 degrees Decreases to 22 degrees by age 1 Increased angle: DDH, neuromuscular disorders Decreased angle: Down syndrome, skeletal dysplasias Perkins' line Vertical line perpendicular to Hilgenreiner line traversing superolateral corner of acetabulum Ossified femoral head should be medial to line Line should bisect middle third of femoral metaphysis Subluxation, dislocation if metaphysis lateral to line Intersection of Perkins and Hilgenreiner lines: femoral head should be in lower inner quadrant Shenton arch Continuous smooth arch connecting medial cortex of proximal metaphysis of femur and inferior edge of superior pubic ramus Discontinuous in DDH, dislocation Other features Abnormal sclerosis of acetabulum Pseudoacetabulum Delayed ossification of femoral head

Developmental dysplasia of hip, radiography (bill)
Wait 2 weeks before performing US
Physiologic ligamentous laxity during first few days of life

Static coronal image
Iliac bone (echogenic line) should bisect femoral head
Normal femoral head covered at least 50% by acetabulum
Alpha angle
Angle between line through iliac bone (baseline) and line along acetabular roof (roofline)
Normally > 60 degrees (55 degrees in newborns)
Shallow angle may be followed to assess
Beta angle
Angle between line through iliac bone (baseline) and line along acetabular labrum (inclination line, cartilaginous roof)
Normally < 55 degrees (less cartilaginous coverage, more bony coverage of femoral head)

Developmental dysplasia of hip, ultrasound (bill)
Nonlethal
Autosomal recessive

Features
Extremities
Rhizomelia
Metaphyseal enlargement
Hitchhiker thumb and great toes
Flexion contractures
Clubfoot
Enlarged ears

Diastrophic dysplasia
Results from failure of recanalization (around 10 weeks)
Incidence: 1:3500 live births
Atresia:stenosis = 2:1

Clinical
Usually presents at birth or within first few days of life
Clinical: bowel obstruction, bilious vomiting

Duodenum is most common site for intestinal atresia
Atresia, stenosis almost always just distal to ampulla of Vater

Double bubble sign
Diagnostic of atresia
UGI series unnecessary
Dilatation of bulb only seen with chronic obstruction
Not enough time to dilate with acute obstruction such as midgut volvulus

Distal gas seen with duodenal stenosis and in Y-shaped biliary-pancreatic duct inserting above and below atresia

Associations
Chromosomal
Down syndrome (30%)
General
Polyhydramnios, prematurity (40%)
GI
Annular pancreas (associated with intrinsic duodenal abnormalities)
Preduodenal portal vein
Malrotation
Other intestinal atresias
Imperforate anus with or without sacral anomalies
Esophageal atresia
Biliary atresia
VACTERL
Congenital heart disease
Renal anomalies

Treatment
Duodenojejunostomy
Duodenoduodenostomy

Duodenal atresia, stenosis (bill)
Obstructing membrane with pinhole
Stretched downstream
Windsock configuration
Associated with trisomy 21
Presents later in life than duodenal atresia
Problems as transition to solid foods
Duodenal web
CRITOE

Capitellum: 1 year
Radial head: 5 years
Internal (medial) epicondyle: 7 years
Trochlea: 10 years
Olecranon: 10 years
External (lateral) epicondyle: 11 years

Elbow ossification centers
Chondroectodermal dysplasia
Autosomal recessive
Amish community
Similar to Jeune syndrome
50% have ASD (unlike Jeune syndrome)
Usually nonlethal (unlike Jeune syndrome)

Features
Extremities
Mesomelia and acromelia
Premature ossification of proximal humeral and femoral epiphysesProximal phalanges longer than distal phalanges
Polydactyly: postaxial (ulnar side)
Abnormal nails
Shortened tubular bones with cone-shaped epiphyses
Early ossification of femoral head
Thorax
Short ribs (like Jeune syndrome)
Ears
Congenital ear anomalies

Pelvis
Decreased acetabular angle
Trident acetabulum (like Jeune syndrome, thanatophoric dysplasia)
Heart
Atrial septal defect (50%, unlike Jeune syndrome)

Ellis-van Creveld syndrome
Congenital muscular torticollis
Age: 2 weeks – 2 years

Clinical
Head tilted toward and turned away from abnormal sternocleidomastoid muscle
Discrete neck mass
Rapidly develops in neonatal period
Spontaneously disappears in 4-8 months

Pathology
Injury to sternocleidomastoid muscle in utero or during birth
May be related to trauma or venous occlusion
Replacement of striated muscle by fibrous tissue

Congenital muscular torticollis
Age: 2 weeks – 2 years

Clinical
Head tilted toward and turned away from abnormal sternocleidomastoid muscle
Discrete neck mass
Rapidly develops in neonatal period
Spontaneously disappears in 4-8 months

Pathology
Injury to sternocleidomastoid muscle in utero or during birth
May be related to trauma or venous occlusion
Replacement of striated muscle by fibrous tissue

Fibromatosis colli
Synonyms
Meconium plug syndrome
Small left colon syndrome
Colonic inertia

Pathology
Ganglionic cell immaturity
Distal colon has abnormal motility
Temporary unlike Hirschsprung’s
Always concern for underlying Hirschprung’s (some centers biopsy all patients)
Unrelated to cystic fibrosis

Associations
Maternal diabetes
Maternal magnesium sulfate for eclampsia

Enema
Multiple filling defects
Small left colon (ascending, transverse colon more dilated)
Rectum normal in caliber (contrast with Hirschprung’s)
Plugs often passed during or shortly after enema (therapeutic; symptoms resolve within hours)

Functional immaturity of colon
Severe vomiting (most common)
Gastroenteritis
Appendicitis

Impaired swallowing
Esophageal atresia
Neurologic impairment
Mechanical ventilation (paralyzed bowel)

Displaced bowel loops
Hernia (CDH)
Omphalocele
Mass
Ascites

Gasless abdomen
Germinal matrix
Site of neuronal precursors in subependymal region
Highly vascular
Located near caudothalamic groove (inferior to lateral ventricles)
Exists only during 24th-32nd week of gestation
Highly vulnerable to hypoxemia and ischemia
Later in pregnancy, mature neuroectodermal cells of germinal matrix migrate to cerebral cortex

Causes of hemorrhage
Trauma: birth
Coagulopathies: ECMO, Rh incompatibility, drugs
Ischemic or hypoxic insult

Risk factors
Prematurity
Low birth weight

Grading
Subependymal hemorrhage
No long-term abnormality
Intraventricular hemorrhage without ventricular dilatation
10% mortality
Intraventricular hemorrhage distending ventricle
20% mortality
Intraparenchymal hemorrhage
> 50% mortality

Germinal matrix hemorrhage, background (bill)
Aging of hemorrhage
Acute (< 7 days): very hyperechoic, no shadowing Subacute, chronic hemorrhage: echogenicity and size decrease within 2-3 weeks Germinal matrix hemorrhage Best seen on sagittal view Most frequent location is anterior to caudothalamic groove Choroid plexus hemorrhage Lumpy-bumpy appearance Normal choroid plexus pulsates; hemorrhage does not Intraparenchymal hemorrhage May occur as sequela of germinal matrix hemorrhage, birth trauma, Rh incompatibility, etc Ultimately results in porencephaly Hematoma becomes hypoechoic within few days to weeks Ventricular dilatation Occurs in 75% after hemorrhage Ventricles should not occupy more than 1/3 of entire hemisphere on coronal scan Serial qualitative assessment of ventricular size more useful than quantitative assessment Rounding of superolateral angles of frontal horns Dilatation of occipital horns
Germinal matrix hemorrhage, ultrasound (bill)
Beta-hemolytic streptococci
Most common type of pneumonia in neonates
Acquired during birth
At least 25% of women are colonized
Premature infants infected more often than term infants

Findings
Bilateral granular opacities, low lung volumes
Looks like hyaline membrane disease
Pleural fluid is distinguishing factor (uncommon in hyaline membrane disease; present in 67% of beta-hemolytic streptococcal pneumonia)

Group B streptococcal pneumonia (bill)
Triad
Acute renal failure
Microangiopathic hemolytic anemia
Thrombocytopenia

Other features
Colitis
CNS manifestations (25-50%)
Rhabdomyolysis
Diabetes

Demographics
Children 1-5 years

Etiology
Infectious agent may be initial stimulus (?endotoxin)
Vascular endothelial damage leads to fibrin strand formation
Red blood cells and platelets damaged
Vascular congestion in multiple organ systems (renal, CNS, GI)

Hemolytic uremic syndrome
Most common benign pediatric liver tumor
Most common symptomatic vascular lesion of infancy
85% < 6 months 50% associated with cutaneous hemangioma Clinical Abdominal mass Hight output CHF (15%) Consumptive coagulopathy (Kasabach-Merritt syndrome) Anemia Jaundice Hemorrhage +AFP in 3% Variable appearance Well-defined or diffuse Most often heterogeneous Prominent vessels Abrupt caliber change of abdominal aorta at celiac axis from arteriovenous shunting MRI, CT characteristics of hemangioma in adults Natural history Proliferative phase in first 18 months of life Tends to involute spontaneously over months-years Serial US: progressive decrease in size and increase in calcification
Hepatic hemangioendothelioma (bill)
Age < 5 years Hepatoblastoma (+AFP) Hemangioendothelioma (+AFP in 3%) Mesenchymal hamartoma Metastatic disease (Wilm's, neuroblastoma) Age > 5 years
Hepatocellular carcinoma (+AFP)
Undifferentiated embryonal sarcoma
Hepatic adenoma
Metastatic disease
Lymphoma
Angiosarcoma

Immunocompromised
Lymphoproliferative disorder
Fungal infection

Nonneoplastic
Abscess (fungal, bacterial, granulomatous)
Hematoma
Cyst

Hepatic mass (<5yrs,>5yrs)
Rare
Benign
Predominantly cystic liver mass
Most commonly presents in infancy (almost always before age 2)
Developmental anomaly (not true neoplasm)

Clinical
Large painless abdominal mass
Normal AFP

Findings
Large multilocular cystic mass
Large cysts (> 10 cm) may be seen
Thin internal septations
Solid component may be predominant: multiple small cysts within may look like Swiss cheese
10% exophytic

Hepatic mesenchymal hamartoma
Most common primary liver tumor of childhood (43% of liver masses)
Infants, younger children (< 3 years) Predisposing conditions Beckwith-Wiedemann syndrome Hemihypertrophy Familial polyposis coli Gardner syndrome Wilms tumor Biliary atresia Presentation: painless mass Increased AFP > 90%
Overall survival: 63-67%

Findings
Well-defined
Displaces rather than invades adjacent structures (i.e. falciform ligament)
Heterogenous: necrosis, hemorrhage
Calcification (50%)
Metastases: lungs > lymph nodes, brain

Hepatoblastoma
Pathology
Absence of myenteric plexus cells (agangliosis, incomplete craniocaudal migration of embryonic neuroblasts) in distal segment of colon
Causes hypertonicity and functional obstruction
Affected colon is small in caliber
Proximal colon is dilated
Involves rectum and variable amount of colon proximally
No skip lesions

Clinical
80% present in first 6 weeks of life with failure to pass meconium, obstruction, intermittent diarrhea and constipation
May present later in life with problems related to constipation
Males >> females (6:1)
Diagnosis: rectal biopsy

Associations
Down syndrome (5%)
Congenital neuroblastoma

Complications
Intestinal obstruction (neonates)
Perforation
Enterocolitis (15%)

Findings
Need early enema filling views of rectum, sigmoid (frontal and lateral)
Abnormal rectosigmoid ratio: sigmoid larger than rectum (rectum should be largest of left colon normally)
Fasciculations, saw-toothed irregularity of denervated segment
May mimic microcolon if entire colon involved
Avoid enema if presenting with colitis
Significant barium retention on 24-hour barium enema

Treatment
Surgical resection of denervated segment (Swenson, Duhamel, Soave operations)
Myomectomy

Hirschprung’s disease (bill)
Surfactant deficient disease, respiratory distress syndrome
Most common cause of death in newborns
Premature infants
Inability of premature type II pneumocytes to produce surfactant
Alveolar collapse, interstitial edema, and noncompliant lungs
Symptoms within first 2 hours of life

Findings
Low lung volumes
Bell-shaped thorax if not intubated
Bilateral granular opacities (collapsed alveoli interspersed with open alveoli)
Larger bronchi do not collapse (prominent air bronchograms)
Severe: coalescence of granular opacities to form diffuse lung opacity
Absent pleural effusions

Signs of prematurity
No subcutaneous fat
No humeral ossification center
Endotracheal tube

Therapy: surfactant administration
Nebulized and aerosol forms
Decreases oxygen and ventilator requirement
Decreases airblock complications
Decreases intracranial hemorrage and bronchopulmonary dysplasia
Increases development of PDA or pulmonary hemorrhage
May have acute desaturation in response to surfactant

Complications
Persistent PDS (signs of CHF)
PIE
Hemorrhage
CHF
Aspiration
Superimposed pneumonia

Hyaline membrane disease (bill)
Dilatation of vagina and uterus proximal to obstruction
Must evaluate GU and GI tract to exclude associated anomalies

Etiologies
Imperforate hymen
Benign
More commonly recognized during early adolescence when menses begins
Vaginal or cervical atresia, septum
Often manifests in neonatal period

Hydrometrocolpos
Jaundice seen at level > 5-10 mg/dL

Types
Unconjugated
Physiologic jaundice: peaks at < 12 mg/dL on day 3 in term infants Hemolysis Conjugated Neonatal hepatitis Biliary atresia Choledochal cyst Inborn errors of metabolism

Hyperbilirubinemia (bill)
Children mask findings of hypovolemic shock longer than adults
More pronounced peripheral vasospasm, tachycardia

CT
Abnormal intense enhancement
Bowel wall
Mesentery
Adrenal glands
Liver
Kidneys
Pancreas
Decreased caliber of aorta and IVC
Diffusely dilated fluid-filled bowel loops
Focal more consistent with bowel wall injury

May be seen before clinical findings of shock
Poor prognosis

Hypoperfusion complex (shock bowel)
Most common diffuse pulmonary hemorrhage syndrome in childhood
Onset between 1 and 7 years of age
May be associated with autoimmune disorders

Pathology
Alveolar hemorrhage
Hemosiderin-laden alveolar and interstitial macrophages
Interstitial thickening, alveolar septal fibrosis
Leads to pulmonary hypertension, cor pulmonale

CT
Lobular or diffuse ground-glass opacities
Smooth interlobular septal thickening

Idiopathic primary pulmonary hemosiderosis (bill)
Viral pneumonia

Bronchopulmonary dysplasia

Hyaline membrane disease

Storage disease
Gaucher’s
Niemann-Pick

Lymphangiectasia
Severe disease, usually fatal by 1-2 years

Langerhans cell histiocytosis

Interstitial lung opacities (6)
Intussusceptum: proximal bowel
Intussuscepiens: distal bowel (receives intussusceptum)

Types
Ileocollic (> 90%)
Ileoileal (more common in adults)
Ileoileocolic
Colocolic

Clinical
Crampy abdominal pain (90%)
Vomiting (90%)
Palpable right-sided abdominal mass (60%)
Currant-jelly stools (60%)
Lethargy

Causes
Idiopathic (90%)
Lymphoid hypertrophy in terminal ileum
Secondary to viral disease
More common in winter and spring
Females > males
Typical age: 3 months – 3 years
Pathologic lead point (10%)
Meckel’s diverticulum
Lymphoma
Inspissated feces (cystic fibrosis)
Bowel wall hemorrhage (Henoch-Schonlein purpura)
Polyp or other tumor
Cyst

Intussusception (bill)
US
Mass with alternating rings of hyper and hypoechogenicity (target or donut sign)
Longitudinal: pseudokidney
Sensitivity: 100%
NPV: 100%

CT
Mass in cecum or ascending colon
Alternating rings of low, high attenuation

Plain film
Rarely normal
Constipation excludes intussusception (cathartic effect on colon)
Paucity of gas in right abdomen
Nonvisualization of air-filled cecum or ascending colon
Meniscus of soft tissue mass (typically in ascending colon or hepatic flexure)
LLD view to see gas in ascending colon
SBO

Intussusception, imaging (bill)
Dilute contrast (50% gastrograffin) placed 3 feet above table top (alternative air reduction)
Avoid abdominal palpation
Maintain constant hydrostatic pressure, wait for three minutes for contrast to pass
Try three times before surgery contemplated
Successful reduction marked by free flow of contrast into terminal ileum
Evaluate for pathologic lead point in patients older than 3 years
Postevacuation and 24 hour films obtained
Contraindications
Free air
SBO
Henoch Schonlein purpura
Peritonitis

Outcome
Successful in 80%

Complications
Recurrence in 5-10% (half within 48 hours after initial reduction)
Perforation (0.5%): occurs at innermost wall (intussusceptum) or outermost wall (intussuscipiens)

Intussusception, reduction (contrast)
Due to in utero ischemia rather than failure of recanalization
Ileum most commonly affected
May involve multiple sites
Associated with polyhydramnios (20-40%)

Findings
Dilated small bowel
Microcolon

Jejunal and ileal atresia
Asphyxiating thoracic dystrophy
Autosomal recessive
Usually lethal
May be indistinguishable from Ellis-van Creveld syndrome
Renal disease in adulthood

Features
Thorax
Short ribs
Respiratory distress
Extremities
Mesomelia and acromelia
Premature ossification of proximal humeral and femoral epiphysesMetaphyseal irrgularity and beaking
Polydactyly
Pelvis
Decreased acetabular angle
Squared iliac wings
Trident acetabulum (also seen in thanatophoric dysplasia, Ellis-van Creveld syndrome)

Jeune syndrome
Congenital anomaly of unknown causes
May be caused by circulatory insult in 6th week of development

Findings
Fusion of two or more cervical vertebral bodies
Scoliosis
Sprengel deformity: elevated, rotated scapula (1/3)
Omovertebral bone: accessory bone extending from spine to medial border of scapula

Associations
Renal anomalies
Cardiovascular anomalies
Synkinesia (mirror movement)
Hearing impairment
Torticollis
Facial asymmetry

Klippel-Feil syndrome
Lead interferes with resorption of primary spongiosa during growth
New bone laid down on top of primary spongiosa
Appears as dense metaphyseal line
Best seen in knee or distal radius where growth is most rapid
Not apparent unless lead > 70-80 g/dL
Lead lines not affected by treatment: disappear spontaneously within 4 years
Similar dense lines seen with other heavy metal poisoning (phosphorus, bismuth)
May cause undertubulation
Lead poisoning
Pathology
Normal 270-degree rotation places duodenaojejunal junction in LUQ and cecum in RLQ
Long fixed base keeps mesentery from twisting
Short base of mesentery predisposes to twisting around SMV, SMA
Other causes of malrotation: Ladds bands (fibrous peritoneal bands), paraduodenal hernia

Clinical
May present at any age: 75% during first 6 weeks of life
Presentation: bilious vomiting (surgical emergency if malrotation present)
Associations
Gastroschisis
Omphalocele
Diaphragmatic hernia
Duodenal or jejunal atresia

Ligament of Treitz
Duodenojejunal junction
Normally to left of spine
Same level or more superior than duodenal bulb
If guidelines not absolutely met, follow contrast through remainder or bowel to ensure jejunal loops in LUQ and cecum in RLQ
Junction is mobile in children: factitiously moved by masses or distended loops
Nasojejunal tube may alter anatomy

Malrotation and midgut volvulus, background (bill)
Plain film
Bowel obstruction
Abnormally positioned bowel loops
Normal cecal position does not exclude malrotation but makes much less likely
Bilious emesis in critically ill infant with complete obstruction on plain film: patient goes directly to surgery

UGI
Abnormal position of duodenojejunal junction because of absent ligament of Treitz
Beaking at site of obstruction
Spiraling of small bowel as it twists around SMA (corkscrew)
Edema of bowel wall
Jejunal loops in right abdomen should raise possibility of diagnosis but not be relied upon as sole criteria

Barium enema
Normal BE excludes 97% of malrotation
Can determine if there is concomitant cecal volvulus

US
Reversal of SMA and SMV relationship (SMV should be to right of SMA): not sensitive or specific
Distended proximal duodenum

CT
Reversal of SMA and SMV relationship
Gastric outlet, duodenal obstruction
Spiraling of bowel loops around superior mesenteric vessels

Malrotation and midgut volvulus, imaging (bill)
Obstruction of distal ileum from abnormally tenacious meconium
Almost exclusively in cystic fibrosis (presenting finding in cystic fibrosis in 10%)
Usually at level of ileocecal valve

Complications
Perforation
Volvulus
Peritonitis
Intussusception

Plain films
Distal obstruction
Soap bubble appearance: air mixed with meconium
Calcification from meconium peritonitis (15%)
Absence of air fluid levels
Water-soluble contrast enema
Microcolon
Distal 10-30 cm of ileum larger than colon
Inspissated meconium in terminal ileum
Hyperosmolar contrast may stimulate passage of meconium

Treatment
Serial water-soluble enemas (attempt to remove nonsurgically; therapeutic in 50-60%)

Meconium ileus
Epidemiology
Most common solid renal mass in neonate
Usually diagnosed in first 3 months of life
Almost all diagnosied before age 2
Uncommon in children and rare in adults

Pathology
Fetal renal hamartoma, stromal hamartoma, leiomyomatous hamartoma
Benign (hamartoma)
Develop from line of metanephric cells that have lost potential for divergent differentiation
Most are nonfunctioning
Composed primarily of mesenchymal connective tissue (cut surface looks like leiomyoma of uterus)

Findings
Solid, very large intrarenal mass
Typically involves renal sinus
Replaces large portion of renal parenchyma
May contain cystic, hemorrhagic, or necrotic regions
Local infiltration of perinephric tissues uncommon
Hypoechoic (cystic) regions

Treatment
Excision for diagnosis not cure
Uncertainty regarding sarcomatous degeneration

Mesoblastic nephroma
Nephrogenic adenofibroma, embryonal adenoma
Benign renal tumor
No age predilection

Clinical
Pain
Hypertension
Hematoma
Flank mass
Hypercalcemia
Polycythemia

US
Well-defined
Solid
May be hypoechoic or hyperechoic
May have cyst with mural nodule

CT
Iso-hyperdense
Small calcifications
Enhances less than normal renal parenchyma

Metanephric adenoma
Stress lines
Neonate
Hypoperfusion of rapidly growing metaphyses of long bones
Causes: fever, congenital heart disease, any severe disease
Leukemia
Lymphoma
Metastatic neuroblastoma
Syphilis
Juvenile chronic arthritis
Metaphyseal lucent lines (6)
Growth recovery lines
Heavy metal poisoning (lead)
Healing rickets
Hypothyroidism
Syphilis
Systemic illness
Metaphyseal sclerotic lines (6)
Delayed maturation of femoral head
Fragmented appearance
No flattening of epiphyses as seen in Legg-Calve-Perthes disease
Ultimately becomes normal
Usually < 5 years (85% < 3 years) Usually bilateral Asymptomatic or mild symptoms Occasional progression to AVN (20%) DDX Legg-Calve-Perthes disease (older child) Hypothyroidism Multiple epiphyseal dysplasia
Meyer dysplasia
Ileal atresia
Meconium ileus
Functional immaturity of the colon
-Maternal diabetes
-Maternal MgSO4
Total colonic Hirschsprung’s disease
Megacystic-microcolon-hypoperistalsis syndrome
Microcolon (5)
Primarily in premature infants
Typically in 1st or 2nd week of life
Less common in older infants under extreme stress (e.g. cardiac surgery)
Mortality up to 30%

Etiologies
Idiopathic: most likely combination of ischemia and infection
Feeding
Possible substrate for growth of organisms
NEC rare in unfed babies

Location
Most commonly affects ileum and ascending colon
Usually patchy involvement

Increased incidence
Premature infants
Neonates with bowel obstruction (e.g. atresia)
Congenital heart disease

Other associations
Infection
Hypoxia
Stress
Hypotension
Hyperosmolar formula feedings (90% of cases)
Hirschprung’s disease
Bowel obstruction

Necrotizing enterocolitis, background (bill)
Perforation

Bowel stricture
Delayed
Most commonly involves descending colon

Postsurgical
Short bowel syndrome
Dumping
Malabsorption

Necrotizing enterocolitis, complications (bill)
Focal dilatation of bowel, especially in RLQ (first finding)
Featureless, unfolded appearing small bowel loops
Separation of bowel loops: suggests bowel wall thickening
Unchanged bowel gas pattern on serial exams
Pneumatosis (most definitive)
Bubble-like, curvilinear lucencies overlying bowel
Looks like stool, but stool-like patterns uncommon in sick premature infants in ICU
Portal venous gas
Free air
Necrotizing enterocolitis, imaging (bill)
Edema
Patent ductus arteriosus

Hemorrhage
Surfactant administration

Worsening surfactant deficiency

Developing neonatal pneumonia

Diffuse microatelectasis
Artifactual secondary to low lung volumes

Neonatal acute diffuse lung consolidation (5)
High lung volumes, streaky perihilar opacities (most born term)
– Meconium aspiration syndrome
– Transient tachypnea of newborn
– Neonatal pneumonia

Low lung volumes, granular opacities (most are premature)
– Hyaline membrane disease
– Beta-hemolytic streptococcal pneumonia

Neonatal diffuse lung disease (5)
Pathology
Multiple foci of primitive renal tissue intermingled with normal renal parenchyma
Primitive metanephric blastema present until 36 weeks gestation (pathologic if present after 36 weeks)
Severe congenital nephroblastomatosis: marked renal enlargement with multifocal areas of nonfunctioning renal tissue
Primitive tissue may cause mass effect with calyceal distortion
Suspect in young patient with renal enlargement

Increased risk of Wilms’ tumors
Wilms’ tumors often contain primitive blastema elements (100% of bilateral Wilms’ tumors)
Multiple foci of nephrogenic rests in up to 25% of kidneys with Wilms’ tumors
Increased risk of multiple and bilateral Wilms’ tumors
Suspicious for Wilms’ if spherical lesion increasing in size with progressive increased heterogeneous enhancement
Requires close follow-up to 7 years of age

Imaging
Usually subcapsular deposits of primitive renal tissue
US: hypoechoic
CT: low-attenuation peripheral nodules with poor enhancement relative to normal renal parenchyma
MRI: low-signal intensity foci on T1 and T2

Nephroblastomatosis (bill)
Most common abdominal malignancy in newborn
3rd most common childhood cancer (leukemia and brain are top 2)
Rare (incidence, 1:30,000)
Arises in neural crest tissue: adrenal medulla (most common), sympathetic chain, organ of Zuckerkandl
Benign counterparts: ganglioneuroma, ganglioblastoma
Children < 2 years Elevated VMA, HVA in urine (90-95%) May cause paraneoplastic syndrome Age-dependent prognosis, pattern of distribution Age < 1 year Better prognosis Spread to liver and skin Age > 1 year
Poor prognosis
Spread to bone

Features associated with better prognosis
Age < 1 year at diagnosis Histologic grade Decreased n-myc amplification Stage IVS Extraadrenal primary

Neuroblastoma, background (bill)
Solid, lobulated tumor
Calcification in 85%
Aggressive, invades adjacent tissues: surrounds and engulfs vessels (Wilms’ displaces vessels)
Surrounds aorta posteriorly, may lift aorta from spine
Readily extends across midline
Lymph node metastases
Neuroforaminal extension (best seen with MRI)
Bright, heterogeneous on T2
Metaiodobenzylguanidine (MIBG) and bone scintigraphy
Neuroblastoma, imaging (bill)
I. Tumor confined to organ of origin
90% survival

II. Extension beyond organ of origin but not across midline
75% survival

III. Crosses midline
30% survival

IV. Disseminated disease
10% survival

IVS. Age < 1 year Metastatic disease to skin, liver, bone marrow (not cortex), lymph nodes Near 100% survival Often no therapy required

Neuroblastoma, staging (Evans)
Diaphyseal fracture
Most common fracture
Indistinguishable from innocent fracture

Highly specific fractures
Posterior rib fracture near costovertebral junction
Subtle prior to callus formation
Metaphyseal corner fracture
Forceful pulling of extremity
Weak zone of provisional calcification susceptible to shearing stress
Bucket handle fracture (corner fracture seen in orthogonal view)

Other specific fractures (three S’s)
Scapular fracture
Spinous process fracture
Sternal fracture

Other fractures
Multiple fractures of different ages, different body parts
Spiral long bone fracture in nonambulatory children
Skull fracture
Clavicle fracture

Periosteal bone formation
Secondary to fracture, subperiosteal hemorrhage, and periosteal elevation

Non-accidental trauma, fractures
Rare benign renal mass
Age: 6 days – 14 months
Most have hematuria as presenting symptom
Boys > girls
Believed to arise from urothelium
Attached to papillary region of renal pyramids
Extends in polypoid fashion into collecting system

Findings
Renal outline maintained
Polypoid filling defects with partial obstruction of collecting system
Calicificaiton (may mimic staghorn calculus which is extremely rare in this age group)

Ossifying renal tumor of infancy
Trauma
Viral inflammation
Sepsis
Idiopathic
Anomaly
Drugs (steroids, etc)
Cystic fibrosis
Hyperlipidemia
Pancreatitis (8)
Physiologic (first 6 months of life)

Prostaglandin E therapy

Vitamin toxicity
Hypervitaminosis A (predilection for metatarsal involvement)
Hypervitaminosis D

Congenital infection
Syphilis
Rubella

Nutritional
Scurvy
Copper deficiency

Non-accidental trauma (subperiosteal hematoma)

Caffey’s disease (infantile cortical hyperostosis; mandible often involved)

Osteomyelitis

Tumor
Neuroblastoma
Osteosarcoma
Ewing’s sarcoma
Leukemia, lymphoma

Infarction
Sickle cell disease

Osteogenesis imperfecta

Periosteal reaction, bill (11)
Necrotizing enterocolitis
Cystic fibrosis
Collagen vascular disease
Leukemia
Milk intolerance
Immunodeficiency
Obstruction
Steroids
Pneumatosis intestinalis (8)
Most common cause of urethral obstruction in male infants
Located in posterior urethra near distal end of verumontanum

Clinical
Obstructive symptoms (35%; hesitancy, dribbling, enuresis)
UTI (35%)
Palbable bladder or kidney in neonates (20%)
Hematuria (5%)
Renal failure: high back pressure, associated reflux

Protective factors
Unilateral vesicoureteral reflux (protects contralateral kidney)
Large bladder or calyceal diverticuli
Ascites in utero
Prognosis
Determined by timing of detection
Diagnosis before 24 weeks carries poor prognosis
Moderate-to-severe upper tract dilatation (pelvis > 10 mm) carries poor prognosis
Echogenic kidneys
Cystic renal parenchymal changes indicating dysplasia

In utero complications
Oligohydramnios
Urine leak (15%): urinoma, urine ascites
Hydronephrosis

Treatment
In utero decompression with vesicoamniotic shunting (has not been shown to improve outcome)
Electrode fulguration after delivery

Posterior urethral valves, background (bill)
US
Thick bladder wall
Bilateral ureteral, collecting system dilatation
Occasionally see dilated posterior urethra inferior to bladder (keyhole in utero)

VCUG
Dilated posterior urethra
Enlarged verumontanum (type I, not III)
Crescentic filling defect bulging into contrast column (type I; spinnaker sail appearance)
Windsock appearance (type III)
Bladder trabeculation, saccules, small diverticuli
Vesicoureteral reflux (50%)

Posterior urethral valves, imaging (bill)
Eagle-Barrett syndrome

Triad
Hypoplasia of abdominal muscles
Widely separated abdominal rectus muscles
Wrinkled appearance of skin looks like prune
Bulging flanks

Cryptorchidism
Bladder distention interferes with descent of testicles

Urinary tract abnormalities
Bilateral severe hydroureteronephrosis
Renal dysplasia (50%)
Trabeculated, hypertrophied bladder: large and distended (hallmark)
Urachal diverticulum, patent urachus
Vesicoureteral reflux common
Hypoplastic prostate (may be underlying etiology)
Scaphoid megalourethra

Prognosis
Lethal in most severe cases
Renal failure in milder cases

Incomplete form (pseudoprune belly syndrome)
Occurs in girls who cannot have cryptorchidism or unilaterally

Prune belly syndrome (bill)
Hypertrophic pyloric stenosis
Idiopathic thickening of pylorus
Progressive gastric outlet obstruction

Epidemiology
Incidence: 1:1000 births
Otherwise healthy infants
Age: 1 week-3 months
Never occurs after 3 months
Males >> females (5:1)
Whites > blacks or Asians
Clinical
Projectile nonbilious vomiting
Palpable olive-sized antral mass
Weight loss
Dehydration
Jaundice
Alkalosis

Associations
Esophageal atresia, TEF
Hiatal hernia
Renal abnormalities
Turner’s syndrome
Trisomy 18
Rubella

Pyloric stenosis, background (bill)
Plain film
Gastric distention (> 7 cm)
Caterpillar sign: peristaltic waves in distended stomach
Decreased air in distal bowel
Thick antral folds
Mottled retained gastric contents

US
Target lesion: hypoechoic muscle with hyperechoic mucosa
Pylorus near GB
Pyloric muscle thickness (single muscular wall) > 3 mm
Pyloric length > 17 mm
Useful as first imaging modality
Limited for exclusion of midgut volvulus

UGI
Delayed gastric emptying
String sign: narrow pyloric channel
Double track sign: puckering of lumen
Shoulder sign: pylorus indents contrast-filled antrum
Mushroom sign: pylorus indents base of duodenal bulb
Beak sign: beaked entrance to pylorus
Remove excess barium with NGT after study

Pyloric stenosis, imaging (bill)
Source of nasal airway obstruction in neonate
Obstructed anterior nasal passage
Thickened anteromedial maxilla
Narrowing of anterior and inferior nasal passage
Lower limit of normal width of pyriform aperture in term infant is 11 mm
Anterior nasal septum may be thinned
Single central megaincisor may be present
Narrow palate with downward hanging bony ridge

Associations
Holoprosencephaly
Encephalocele
Dandy Walker malformation
Hypopituitarism

Pyriform aperture stenosis (bill)
Autosomal recessive polycystic kidney disease (empty bladder)
Glomerulonephritis (CMV)
ATN
Renal infiltration
Glycogen storage disease
Diabetes
Renal vein thrombosis
Leukemia (only malignancy causing this appearance)
HIV
Kawasaki disease
Alport syndrome
Renal echogenicity, (11) (bill)
Pyelonephritis, focal nephronia
ARPKD
ADPKD
Medullary cystic kidney
Late renal vein thrombosis
Loss of corticomedullary differentiation (5)
Pyogenic infection of retropharyngeal space
Cellulitis more common than discrete abscess
Usually follows recent pharyngitis or upper respiratory tract infection
Organisms: S. aureus, Streptococcus B, oral flora
Age: 6-12 months

Clinical
Sudden onset fever, stiff neck
Dysphagia
Occasional stridor

Findings
Widened retropharyngeal space (most common): should normally be less than 1 or ¾ vertebral body width at C1-4
Apex anterior convexity of retropharyngeal soft tissues
Air in soft tissues: only X-ray feature specific for abscess
Straightening of cervical lordosis
CT helpful for defining superior and inferior extent of disease and identifying abscess

Retropharyngeal cellulitis, abscess
Inflammation
Cellulitis
Abscess
Mononucleosis
Tuberculosis

Edema
Hypothyroidism

Trauma

Foreign body

Tumor
Lymphoma
Leukemia
Neuroblastoma
Neurofibroma

Vascular
Hemangioma
Lymphangioma

Lymphadenopathy

Retropharyngeal soft tissue thickening
Originates in renal sinus (unlike Wilms’ which arises in renal cortex)
2% of renal tumors in early childhood
Age: 6 months – 9 years (peak age: 6-12 months)
Poor prognosis
Common metastases to lung, liver, brain
Associated with primary brain tumors: medulloblastoma, ependymoma, glioma, PNET

Findings
Difficult to distinguish from Wilms’ tumor by imaging alone
Peripheral subcapsular fluid collection adjacent to solid tumor lubules (70%)
Centrally located renal mass with subcapsular fluid collection + posterior fossa mass

Rhabdoid tumor
Relative or absolute insufficiency of vitamin D

Vitamin D pathway
Vitamin D3 in food
Skin: conversion of 7-dehydrocholesterin to cholecalciferol
Liver: conversion to 25-OH cholecalciferol
Kidney: 1,25 (OH2) cholecalciferol (active vitamin D)

Causes of vitamin D deficiency
Dietary deficiency (common)
Intestinal malabsorption
Skin disease
Liver disease
Renal tubular acidosis
Renal failure (loss of calcium)
Lack of end-organ response

Insufficient conversion of growing cartilage into mineralized osteiod: buildup of nonossified osteoid
Manifestations most prominent in rapidly growing bones
Survey with frontal views of wrists and knees

Common locations
Costochondral junctions
Distal femur
Both ends of tibia
Proximal humerus
Distal radius and ulna

Rickets, background (bill)
Infantile rickets (6-18 months)
Physeal widening, irregularity
Metaphyseal fraying, cupping
Bowing deformities
Softening of cranial vault (craniotabes)
Delayed closure of fontanelles
Delayed appearance of ossification centers
Rachitic rosary: enlargement of cartilage at costochondral junction

Vitamin D-resistant rickets (> 2 years)
Marked bowing of extremities
Bones may occasionally appear sclerotic
Sclerosis may be seen in SI joints and acetabula

Other findings
Osteomalacia: unsharp, smudged trabecular markings
Looser’s zones or milkman’s fractures: transverse fractures on concave side of long bones
SCFE association
Periosteal reaction: severe rickets

Healing rickets
Ossification of unmineralized osteoid
Zone of provisional calcification: wide band that narrows physis to normal thickness
Periosteal new bone: ossification of subperiosteal osteoid

Rickets, imaging (bill)
Choledochal cyst
Enteric duplication cyst
Mesenteric cyst
Lymphangioma
Dermoid, teratoma
Hydronephrosis
Ovarian cyst
Abscess
Meconium pseudocyst
Right upper quadrant cystic mass (9)
Displacement of femoral head relative to femoral neck through open growth plate
Slippage between proliferative and hypertrophic zones of growth cartilage (distinguish from Salter I fracture which occurs between hypertrophic and provisional calcification zones of cartilage)
Predisposing factor: renal osteodystrophy
Bilateral in up to 50%

Demographics
Males > females (2.5:1)
African Americans
Obese teenagers: 8-17 years (11 years in girls, 14 years in boys)

Clinical
Pain (90%)
History of trauma (50%)

Treatment
Fixation of femoral epiphysis with pins, bone pegs (fixed in slipped position; moving epiphysis risks AVN)

Findings
Slippage of head: posterior and medial
Most prominent on frogleg lateral view
Ice-cream cone
Widened physis (asymmetric)
Line through lateral cortex of metaphysis on frogleg lateral should bisect portion of ossified epiphysis; slippage is medial to this line
Indistinct metaphyseal margin of physis
Decreased height of epiphysis (slips posterior)
Decreased angle of femoral neck anteversion on CT

Complications
Osteonecrosis
Chondrolysis (cartilage necrosis from pins)
Varus deformity
Degenerative OA

Slipped capital femoral epiphyses, background (bill)
Findings
Hindfoot valgus
Normal AP and lateral talocalcaneal angles are 40 degrees
AP talocalcaneal angle increased in hindfoot valgus (opposite clubfoot deformity)
Metatarsus adductus
Base of 4th metatarsal medial to AP axis of calcaneus

Treatment
Frequently requires surgical correction

Skew foot
Chondrodysplasia punctata
Warfarin embryopathy
Alcohol embryopathy (lower extremities)
Zellweger syndrome (patellae)
Stippled epiphyses (4)
Chronic infection caused by Treponema pallidum (spirochete)
Congenital infection by transplacental migration of organism

Clinical
Hepatosplenomegaly
Skin rash
Lymphadenopathy
Anemia

Findings
Osteochondritis
Symmetric involvement at sites of enchondral ossification (growth plates of long bones, costochondral junctions, flat bones, spine)
Lucency and osseous irregularity
Wimberger’s sign: localized destruction of medial portion of proximal tibial metaphysis
Sawtooth metaphysis: metaphyseal serration
Lucent metaphyseal bands

Diaphyseal osteomyelitis
Focal regions of osteolysis
Exuberant periostitis
Saber shin deformity (anterior bowing of tibia)
Periosteal reaction
May be secondary to subperiosteal infection, osteomyelitis, or osteochondritis
Gummas
Tertiary syphilis
Sterile bone abscesses

Syphilis, background (bill)
Most common lethal dysplasia
Sporadic
Polyhydramnios (75%)
Nonimmune hydrops

Features
Extremities
Rhizomelia
Metaphyseal flaring
Telephone receiver femurs (type 1 only)
Skull
Enlarged skull
Frontal bossing
Cloverleaf skull (trilobed): key finding (15%), type 2 only
Spine
Platyspondyly
Thorax
Short ribs
Pelvis
Squared iliac wings
Trident acetabulum
Decreased acetabular angle

Thanatophoric dysplasia
Vascular ring
Tracheomalacia
Congenital stenosis
Paratracheal cyst or mass
Tracheal narrowing (4)
Bacterial tracheitis, membranous croup, membranous laryngotracheobronchitis
Uncommon, potentially life-threatening
Purulent infection of trachea with exudative plaques
Older than croup patients (>3 years)
More ill than patients with croup
Organisms: Staphylococcus aureus, multimicrobial
May be primary bacterial or secondary bacterial after viral infection

Findings
Plaque-like irregularity, loss of definition of tracheal wall
Symmetric or asymmetric subglottic narrowing in child who is too old to have croup
Sloughed membrane may occlude airway

Treatment
Endoscopy with stripping of exudative membranes
Elective endotracheal intubation

Tracheitis, exudative
Variable length of atresia
Usually at junction of proximal and middle thirds of esophagus
May occur in absence of TEF

Findings
Inability to pass NG tube
Gas-filled dilated proximal esophageal pouch
Gasless abdomen rules out fistula (may still have esophageal atresia)
Excessive air in stomach
Aspiration pneumonia

Diagnosis
Pass 8 Fr feeding tube through nose to level of atresia
Distal end of tube marks level of atresia (inject air if necessary)
Injection of 1-2 mL of liquid barium
DDX
Traumatic perforation of posterior pharynx
Pharyngeal pseudodiverticulum

Repair
Done contralateral to aortic arch
Document side of arch

Complications after repair
Recurrent fistula
Esophageal leak immediately postoperative (extrapleural collection suggests leak)
Stricture
Dysmotility
Gastroesophageal reflux

Tracheoesophageal fistula and esophageal atresia, background (bill)
VACTERL
Vertebral anomalies
Anal atresia
Cardiac abnormalities
Tracheoesophageal fistula
Renal anomalies
Limb (radial array) anomalies

Cardiac anomalies
VSD
PDA
Right aortic arch
Other atresias
Duodenal atresia
Imperforate anus

Chromosomal
Trisomy 18
Trisomy 21

Tracheoesophageal fistula and esophageal atresia, associations (bill)
Toxic synovitis
Most common nontraumatic cause of acute limp in child
Nonspecific inflammatory reaction
Unknown etiology
Diagnosis of exclusion
Average age: 3-10 years
Males > females (2:1)
Develop limp over 1-2 days with pain in hip, thigh, and knee
History of recent viral illness in 65%, mild fever in 25%
Complete resolution within few weeks seen with non-weightbearing treatment

Plain film
Usually normal
Joint effusion and regional osteoporosis may be present
Joint space widening (difference > 1 mm)

Transient synovitis of the hip
Wet lung syndrome, transient respiratory distress
Delayed clearance of fetal pulmonary fluid
Clearance facilitated by thoracic squeeze during vaginal deliveries

Components of normal pulmonary fluid clearance
Bronchial squeezing during delivery (30%)
Absorption by lymphatics and capillaries (30%)
Suction (30%)

Causes
Caesarian section (thoracic squeeze bypassed; most common cause)
Maternal sedation (no thoracic squeeze)
Maternal diabetes
Hypoproteinemia
Hypervolemia
Erythrocythemia

Natural history
Benign course (hallmark)
Respiratory distress develops within 6 hours, peaks at 1 day, and resolves in 2-3 days

Findings (looks like pulmonary edema)
Airspace opacities
Coarse interstitial markings
Prominent, indistinct pulmonary vasculature
Fluid in fissures
Pleural effusion
Cardiomegaly

Transient tachypnea of the newborn (bill)
Passes from umbilical artery into pelvis into iliac artery
Turns cephalad into aorta
May thrombose: do not leave tip near major branches
Do not position between T10 and L3

Two desired locations
High: level of descending thoracic aorta (T8-10)
Low: below renal arteries (below L3)

Umbilical arterial catheter
Traverses umbilical vein into portal sinus (junction of left and right portal vein)
Enters ductus venosus (closed at 96 hours) and into IVC with tip at junction with RA (ideal location)
May be misplaced into portal or hepatic venous branch
Complications: liver hematoma, abscess
Umbilical venous catheter
Rare
Steep axis of talus
Normal talocalcaneal angle is 40 degrees on AP and lateral views (intersecting long axes of talus and calcaneus)
Talocalcaneal angle increased in congenital vertical talus (hindfoot valgus)
Dorsal dislocation of navicular on talus
Navicular may become wedge-shaped
Forefoot pronated
Plantar surface flattened or convex: may lead to rocker-bottom deformity
Anterior and middle facets of subtalar joint absent or replaced by fibrous tissue
Isolated deformity in 50%
Associations
Congenital
Neurofibromatosis
Meningomyelocele
Arthrogryposis
Trisomies 13 and 18
Acquired
Cerebral palsy
Poliomyelitis
Spinal muscular atrophy
Overcorrection of clubfoot deformity
Vertical talus, congenital
Epidemiology
Most common renal malignancy in children
8% of childhood malignant tumors
3rd most common renal mass (after hydronephrosis and MCDK)

Age
Extremely rare in newborn
50% < 2 years 80% < 5 years Peak incidence: 3 years Clinical May be asymptomatic Palpable abdominal mass (90%, 12 cm mean diameter at diagnosis) Pain (35%), particularly if intratumoral hemorrhage present Hypertension (50%) Hematuria (5%) Fever (15%) Anorexia (15%) Pathology Embryonal neoplasm: arises from metanephric blastema Large mass arising from cortex of kidney (mean 12 cm) Usually exophytic growth with pseudocapsule Cystic areas: hemorrhage, necrosis Calcification uncommon (15%): curvilinear and amorphous Hyperechoic, hypodense T1-hypointense, T2-hyperintense Hypoenhancing relative to renal parenchyma Invades renal vein and IVC (5-10%; unlike neuroblastoma Displaces vessels; passes anterior to aorta Metastases to liver and lung Bilateral in 5-10% (diagnostic of nephroblastomatosis) Multifocal in 10% Nephroblastomatosis is precursor of Wilms' tumor

Wilms tumor, background (bill)
WT1 gene: chromosome 11p13
WAGR syndrome
Wilms’ tumor
GU abnormalities
Aniridia
Mental retardation
Drash syndrome
Wilms’ tumor
Male pseudohermaphroditism
Progressive glomerulonephritis
WT2 gene: chromosome 11p15
Beckwith-Wiedemann syndrome
Macroglossia
Omphalocele
Visceromegaly
Hemihypertrophy
Wilms tumor, associated syndromes
1 Tumor limited to kidney with intact capsule, completely excised
2 Tumor extending beyond kidney but completely excised
3 Residual tumor confined to abdomen
4 Hematogenous metastases (lung, liver, bone, brain)
5 Bilateral renal involvement
Wilms tumor, staging
Intrasutural bones (named after Worms, a Danish anatomist)
Most commonly associated with lambdoid suture
Normal finding until 1 year

PORKCHOPS
Pyknodysostosis
Osteogenesis imperfecta
Rickets (in healing)
Kinky hair syndrome
Cleidocranial dysplasia
Hypothyroidism
Otopalatodigital syndrome
Pachydermoperiostitis
Syndrome of Down

NOCK UP
Normal
Osteogenesis imperfecta
Cleidocranial dysplasia
Kinky hair
Unusual conditions such as hypothyroidism
Pyknodysostosis

Wormian bones
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