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60 Cards in this Set

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Neuroblastoma - staging, survival by stage, age, and location
Terminology

* Malignant tumor of primitive neural crest cells
* Most common extracranial solid malignancy in children
* 3rd most common pediatric malignancy

Imaging Findings

* Most commonly arises from adrenal gland but can arise anywhere along sympathetic chain from neck to pelvis
* Aggressive tumor with tendency to invade adjacent tissues
* Surrounds and engulfs, rather than displaces vascular structures such as celiac artery, superior mesenteric artery (SMA), and aorta
* Tendency to invade into spinal canal via neuroforamina
* Important to recognize for presurgical planning
* Metastasizes most commonly to liver and bone
* Low attenuation liver lesions or diffuse, heterogeneous liver enlargement
* Destructive bony lesions
* Prognosis and patterns of disease dependent on age
* < 1 year of age: Better prognosis, mets to liver and skin
* > 1 year of age: Worse prognosis, mets to bone
* Often demonstrate calcifications (74% by CT)

Pathology

* Continuous spectrum with more benign counterparts: Ganglioneuroma and ganglioneuroblastoma (determined by degree of cellular maturation)
Wilms - staging
Terminology

* A malignant tumor of primitive metanephric blastema
* Most common abdominal neoplasm in children 1-8 years old

Imaging Findings

* Best diagnostic clue: Large heterogeneous mass replacing kidney and extending into renal vein and inferior vena cava (IVC)

Top Differential Diagnoses

* Neuroblastoma
* Multilocular cystic nephroma
* Clear cell sarcoma and rhabdoid tumor of the kidney
* Renal cell carcinoma
* Nephroblastomatosis
* Congenital mesoblastic nephroma

Pathology

* I: Confined to kidney, completely excised
* II: Local extension, completely resected
* III: Incomplete resection, no distant metastases
* IV: Distant metastases to lung, liver, brain, or bone
* V: Bilateral synchronous tumors

Clinical Issues

* 80% of cases in children less than 5 years old
* Cure rate for Wilms tumor is better than 90% with current therapy
Lucent Metaphyses - DDX
stress lines from any severe dz
> 2 yrs consider neuroblast mets, lymphoma, leukemia
Rickets - physiology
Terminology

* Nutritional rickets: Simple vitamin D deficiency due to lack of vitamin D in diet or inadequate exposure to sunlight is most common cause; calcium deficiency a cause in Turkey and in both tropical and subtropical Africa (rare elsewhere)

Imaging Findings

* Best diagnostic clue: Widened long-bone physes with cupped, splayed, and frayed metaphyses
* Healing: Increased density in zone of provisional calcification seen after treatment in 2-3 weeks with nutritional rickets and 2-3 months with renal rickets

Top Differential Diagnoses

* Leukemia
* Congenital syphilis
* Gymnast wrist

Pathology

* Normal bone development depends upon ready availability of calcium, phosphorus, and vitamin D
* Rickets: Calcification of cartilage and osteoid does not occur → widening of the zone of provisional calcification, which is perceived as physeal widening on radiographs
* Rickets: Metaphyseal widening and cupping caused by stress at sites of ligament attachments, splaying of cartilage cells peripherally, and microfracturing of primary spongiosa by protrusion of physeal cartilage

Clinical Issues

* Most cases of rickets respond to vitamin D therapy and, if necessary, calcium supplementation
Duplicated renal collecting systems
Terminology

* Renal collecting system split into separate upper and lower pole moieties

Imaging Findings

* Best diagnostic clue: Dilatation of upper pole collecting system + ureterocele is diagnostic
* Morphology:
* Upper and lower pole moieties separated by band of renal parenchyma
* Upper pole drained by ectopic ureter
* Lower pole drained by normotopic ureter
* Ectopic ureter inserts inferior and medial to normotopic ureter, in trigone of bladder
* Evaluate kidney in both transverse and longitudinal planes
* Transverse views alone can lead to erroneous diagnosis of ureteropelvic junction (UPJ) obstruction

Top Differential Diagnoses

* Ureteropelvic junction (UPJ) obstruction
* Reflux

Pathology

* Gynecological abnormalities in 50% of affected females

Clinical Issues

* Excellent prognosis with early correction
* In utero treatment not usually indicated
* Complete work-up after delivery

Diagnostic Checklist

* Always evaluate for duplication in presence of hydronephrosis (unilateral or bilateral)
Osteomyelitis
especially presenting in corner of chest film eg. humerus

Imaging Findings

* Best diagnostic clue: Aggressive destructive lesion in metaphysis of child < 5 years old
* Long-bone metaphyses 70% (femur > tibia > humerus > fibula), short bones 6%, pelvis 5%, spine 2%
* Pelvis: Symptoms mimic urinary tract infection, septic hip, acute abdomen, radiculitis
* Earliest finding: Soft tissue swelling next to bone
* CT: Bone destruction, intramedullary gas and fat-fluid level, periosteal reaction, sequestrum, involucrum
* Well-defined areas that do not enhance with gadolinium: Suspect necrosis or abscess formation
* Grayscale Ultrasound: Excludes/includes pyarthrosis, shows soft tissue swelling, also periosteal thickening, hyperemia and elevation due to subperiosteal abscess
* Bone scan: Positive 24-72 hours, demonstrates multiple sites
* Best imaging tool: MR best choice when abscess recognition will dictate need for surgery

Pathology

* Tendency to occur in metaphyses or metaphyseal equivalents (bone next to cartilage, e.g., calcaneal apophysis and acetabulum)
* Staphylococcus (S) aureus commonest (43%), then β-hemolytic streptococcus (S) (10%) and S. pneumoniae (10%)

Clinical Issues

* Incidence 1-3:1,000 in neonatal intensive care
Focal Lung Opacity - Round Pneumonia vs tumor
Terminology

* Bacterial pneumonia with a very round, well-defined appearance on chest radiography, simulating a mass

Imaging Findings

* More common in lower lobes
* Reported size varies between 1-7 cm
* Air bronchograms
* Respects lobar anatomy without crossing fissures
* If child has symptoms of pneumonia and "round" density on chest radiograph, additional imaging with modalities such as CT not necessary
* Follow-up radiograph several weeks after antibiotic therapy may be helpful to document resolution of the process
* This is one of the few indications concerning pneumonia in children where a follow-up chest radiograph may be indicated even if the child becomes asymptomatic in order to exclude underlying mass

Pathology

* In children, collateral pathways of air circulation are not well-developed until approximately 8 years of age
* After 8 years of age, if round mass seen on chest radiograph, high suspicion for other pathology
* Most commonly seen with streptococcal pneumoniae infection

Clinical Issues

* Cough and fever
* May present with abdominal pain
Focal Lung Opacity - CDH vs CAM vs Cong Lobar emphysema vs sequestration:
Congenital cystic adenomatoid malformation (CCAM)

* Appears as multicystic, air-containing mass
* CCAM more likely to have air-fluid levels than CDH
* Appearance does not change in position with CCAM over serial films, can with CDH
* Position of support apparatus altered with CDH

Congenital lobar emphysema (CLE)

* Hyperlucent lung as compared to air filled bowel in CDH
* Often involves upper lobe vs. originating from below in CDH

Pneumonia complicated by cavitary necrosis

* Usually not in neonatal period
* Cysts surrounded by opacified lung



Sequestration

* Typically do not contain air during the neonatal period
* Systemic arterial supply to mass demonstrated
* Only become air-filled when infected
* Usually in left lower lobe

Congenital diaphragmatic hernia (CDH)

* Appears as multicystic, air-containing mass
* CCAM more likely to have air-fluid levels than CDH
* Appearance does not change in position with CCAM over serial films, can with CDH
* Position of support apparatus altered with CDH (i.e. nasogastric (NG) tube, umbilical venous catheter (UVC)) can help with diagnosis NG tube
* Lack of bowel gas in abdomen
Dbl bubble DDx
duodenal atresia:

Terminology

* Most common upper bowel obstruction in neonate

Imaging Findings

* Best diagnostic clue: "Double bubble"
* In the region of the Ampulla of Vater
* Duodenal obstruction
* Must see duodenal jejunal junction (DJJ) to exclude volvulus
* If "double bubble" on x-ray, no further study required
* If distal gas, upper GI

Top Differential Diagnoses

* Malrotation and Midgut Volvulus: Extrinsic
* Duodenal Web or Diaphragm: Intrinsic
* Other Anomalies Associated with Intrinsic Duodenum Obstruction
* Annular pancreas in 33%
* Preduodenal portal vein

Pathology

* Failure of vacuolization (recanalization)
* 30% with DA have Down syndrome (trisomy 21)
* Malrotation: 28%
* Annular pancreas: 33%
* Other associated anomalies: Intestinal atresias; congenital heart disease; anorectal, biliary, renal anomalies; absence of gallbladder; situs anomalies, preduodenal portal vein
* Type I: Most common in DA

Clinical Issues

* Onset of vomiting within hours of birth
* Duodenoduodenostomy most common operation
Prox. small bowel obstruction DDX
NEONATES-
small bowel atresia/stenosis
midgut volvulus
Ladd’s bands

CHILDREN–
intussusception
incarcerated inguinal hernia
perforated appendicitis
Distal Small bowel obstruction DDx
No microcolon: Meconium plug (small left colon) syndrome

* Benign reversible functional obstruction
* Resolves: Often after contrast enema
* R/S ratio usually > 1
* Abrupt transition zone at splenic flexure
* Differentiation made on rectal biopsy




Hirschsprung Disease

* Rectum smaller than sigmoid diameter, serrated mucosa
* Differential diagnosis meconium plug syndrome
* Broad cone-like zone of transition if near splenic flexure

Ileal Atresia

* Microcolon
* Portions of ileum opacified are collapsed and blind ends
* Cannot opacify dilated loops (proximal to atresia)

Meconium Ileus

* Microcolon
* Distal ileum filled with meconium pellets
* Small bowel proximal to meconium filled segment is dilated
* Nearly all have cystic fibrosis

Colonic Atresia

* Microcolon to level of atresia
* Very rare
* Distal obstruction with a single disproportionately dilated loop

Midgut Volvulus

* Normal caliber colon on enema: Perform upper GI
* Mimics distal obstruction: Late presentation
* Dilated bowel loops due to ischemia not obstruction
* Upper GI shows
o Abnormal duodenal jejunal junction
o Obstruction at 2nd to 3rd portion duodenum
o Corkscrew, spiral appearance
Colonic obstruction
No microcolon: Meconium plug (small left colon) syndrome

* Benign reversible functional obstruction
* Resolves: Often after contrast enema
* R/S ratio usually > 1
* Abrupt transition zone at splenic flexure
* Differentiation made on rectal biopsy

No microcolon: Allergic (milk allergy) colitis

* Onset in first weeks of life
* Usually associated with formula feeding
* Enema shows R/S ratio < 1 and sometimes colitis
* Eosinophilia on rectal biopsy with normal ganglion cells

Microcolon (not total colonic HD): Meconium ileus

* Meconium pellets obstructing terminal ileum

Microcolon (not total colonic HD): Ileal atresia

* Abrupt cutoff of contrast in terminal ileum

Microcolon (not total colonic HD): Immature colon

* Premature infant with small colon throughout

Microcolon (not total colonic HD): Colonic atresia

* Small colon which usually ends abruptly in left colon
Duodenal atresia
Terminology

* Most common upper bowel obstruction in neonate

Imaging Findings

* Best diagnostic clue: "Double bubble"
* In the region of the Ampulla of Vater
* Duodenal obstruction
* Must see duodenal jejunal junction (DJJ) to exclude volvulus
* If "double bubble" on x-ray, no further study required
* If distal gas, upper GI

Top Differential Diagnoses

* Malrotation and Midgut Volvulus: Extrinsic
* Duodenal Web or Diaphragm: Intrinsic
* Other Anomalies Associated with Intrinsic Duodenum Obstruction
* Annular pancreas in 33%
* Preduodenal portal vein

Pathology

* Failure of vacuolization (recanalization)
* 30% with DA have Down syndrome (trisomy 21)
* Malrotation: 28%
* Annular pancreas: 33%
* Other associated anomalies: Intestinal atresias; congenital heart disease; anorectal, biliary, renal anomalies; absence of gallbladder; situs anomalies, preduodenal portal vein
* Type I: Most common in DA

Clinical Issues

* Onset of vomiting within hours of birth
* Duodenoduodenostomy most common operation
Midgut Volvulus
Terminology

* Malrotation: Abnormal fixation of small bowel mesentery resulting in short mesenteric base that is prone to twisting
* Volvulus: Abnormal twisting of small bowel about the superior mesenteric artery that can result in bowel obstruction and bowel ischemia/necrosis

Imaging Findings

* Abnormal position of cecum by small bowel follow through or barium enema
* Volvulus: Cork screw or "Z"-shaped appearance of the duodenum which does not cross to the left of midline
* Diagnosis of malrotation made on upper GI when criteria for normal position of DJJ (ligament of Treitz) are not met
* DJJ is at the same level or more superior than duodenal bulb
* On lateral view, duodenum typically courses posteriorly, then inferiorly

Pathology

* With normal embryonic rotation, both the duodenojejunal and ileocolic portions of the bowel rotate counterclockwise 270° around the axis of the omphalomesenteric vessels
* Etiology: Development anomaly resulting in narrow mesenteric pedicle secondary to abnormal fixation and rotation of bowel

Clinical Issues

* Classic presentation: Bilious vomiting
meconium ileus
Terminology

* Neonatal obstruction of the distal ileum due to abnormally thick, tenacious meconium
* Essentially all patients with MI have cystic fibrosis (CF)

Imaging Findings

* Best diagnostic clue: Distal bowel obstruction with microcolon and meconium-filled terminal ileum (TI) on enema
* Multiple dilated bowel loops
* ± Bubbly lucencies right lower quadrant (RLQ)
* Few, if any air-fluid levels (sticky meconium)
* Soft tissue mass or gasless abdomen
* Curvilinear calcifications on peritoneal surface or lining pseudocyst
* Smallest of microcolons
* Dilated, thick-walled, echogenic bowel loops
* Best imaging tool: Water-soluble enema

Top Differential Diagnoses

* Ileal Atresia
* Total Colonic Hirschsprung Disease (HD)

Pathology

* Mutations in CFTCRG, faulty electrolyte transport across epithelium

Clinical Issues

* Most common signs/symptoms: Failure to pass meconium, abdominal distention, bilious emesis
* Uncomplicated MI: Serial hyperosmotic, water-soluble enemas vs. surgery
* Complicated MI: Surgery
meconium peritonitis
Terminology

* Chemical peritonitis with associated inflammatory response from in utero bowel perforation and peritoneal leakage of sterile meconium

Imaging Findings

* Abdomen radiograph showing linear, curvilinear, or punctate peritoneal calcifications
* Ultrasound showing peritoneal calcifications and/or pseudocyst ± rim calcification
* Calcifications; punctate or linear
* If prenatal US suggestive, post-natal abdomen x-ray
* Contrast fluoroscopic study to limit differential diagnosis

Top Differential Diagnoses

* Cystic Fibrosis: Meconium Ileus
* Intestinal atresia
* Malrotation with midgut volvulus
* Intussusception
* Mesenteric/internal bowel hernias
* Congenital bands
* Viral Infections

Clinical Issues

* Peritoneal calcifications on prenatal or neonatal imaging
* Abdominal distention
* Failure to pass meconium after birth
meconium plug syndrome
Terminology

* Meconium plug syndrome (MPS), small left colon syndrome (SLCS), functional immaturity of the colon (FIC)
* Transient functional obstruction of the newborn colon
* Common cause of distal neonatal bowel obstruction

Imaging Findings

* Multiple dilated bowel loops in neonate
* Small left colon to the splenic flexure
* Abrupt zone of transition to dilated proximal colon at the splenic flexure
* Multiple filling defects may fill left colon, but not required
* Differential diagnosis long segment Hirschsprung disease
* Best imaging tool: Water-soluble contrast enema

Pathology

* Distal colon spastic and narrowed, causes functional obstruction usually at splenic flexure
* Genetics: No association with cystic fibrosis
* Etiology: Probably immaturity of ganglion cells or hormonal receptors
* Associated abnormalities: Most with MPS do not have associated abnormalities

Clinical Issues

* Abdominal distention
* Delayed or failed passage of meconium (> 24-48 hours)
* Bilious emesis
* Condition resolves over time, hastened by enemas
Anal rectal atresia
Terminology

* Anorectal malformation (ARM), imperforate anus, cloacal malformation

Imaging Findings

* Multiple dilated bowel loops; clinically no anal opening
* Distal bowel obstruction, intraluminal calcification
* Bladder findings
* Normal; vesicoureteral reflux (VUR); wall-thickening; neurogenic bladder; fistula to rectum
* Urethral findings
* Opens to cloaca, fistula from rectum to posterior > bulbous > penile urethra
* Vaginal findings
* Normal; rectovaginal fistula; congenital anomalies (septate, duplicated, etc.)
* Document connection to urethra, bladder, or vagina
* Pelvic musculature, neorectum, and anal sphincter position/integrity
* Ultrasound (US) hypoechoic rectal pouch-perineal distance

Pathology

* Currarino triad (ARM, sacral deformity, presacral mass)
* Classification: Location of pouch relative to levator ani muscle (PCL on lateral pelvis)

Clinical Issues

* Most common signs/symptoms: Absent/abnormal anal opening, failure to pass meconium, meconium per vagina or urethra
Hirschprung's
Terminology

* Functional obstruction of the bowel due to lack of intrinsic enteric ganglion cells

Imaging Findings

* Best diagnostic clue: Recto-sigmoid (R/S) ratio < 1 on contrast enema
* Denervated colon is small, narrow, spasmodic
* Innervated more proximal bowel is dilated
* Multiple loops of dilated bowel
* Transition zone from abnormally small distal to dilated proximal colon
* Neonates: Water-soluble contrast enema
* Infants and children: Barium enema
* Critical views: Lateral and AP of Rectosigmoid colon during early filling

Pathology

* Absence of both myenteric and submucosal plexus
* Most cases sporadic
* Familial HD in about 8-10% of HD
* Defective craniocaudal migration of vagal neural crest cells 5-12 weeks gestation

Clinical Issues

* Failure to pass meconium 24-48 hours
* Abdominal distention
* Bilious vomiting
* Constipation since birth
* 90% diagnosed in newborn
* Untreated, HD can lead to toxic megacolon, enterocolitis, sepsis and death
* Surgical resection of affected colon
Intusseception - demographics, causes, techniques for reduction, contraindications
Terminology

* Intussusception: Forward peristalsis results in invagination of more proximal bowel (the intussusceptum) into lumen of more distal bowel (the intussuscipiens) in a telescope-like manner

Imaging Findings

* Best diagnostic clue: Radiography: Meniscus of soft tissue mass outlined in air-filled colon
* Most common site: Terminal ileum/ileocecal valve

Pathology

* Bowel wall congestion from venous obstruction may lead to bowel ischemia and necrosis
* Seasonal occurrence (winter, spring) with viral illnesses

Clinical Issues

* Alternating lethargy and irritability
* Colic
* Most common between 3 months-1 year of age
* Surgery reserved for cases of reduction failure
* Success rates 80-90% with air reduction
* Risk of perforation 0.5%

Diagnostic Checklist

* Left-side-down decubitus/prone views can be helpful in showing lack of air-filled cecum
* CT: Colonic mass with alternating rings of high and low attenuation
* May not be located in RLQ if intussusception has progressed distally

Treatment

* Imaging guided pressure reduction is treatment of choice
* Surgery reserved for cases of reduction failure
* Air insufflation or liquid contrast with fluoroscopic guidance most common methods
* Hydrostatic reduction under ultrasound guidance
* Contraindications: Peritonitis - exam, free peritoneal air - radiography (rare)
* Findings associated with decreased success rate but not contraindications
o Small bowel obstruction
o Prolonged history of symptoms (days)
o Poor clinical condition: Lethargy
* Preparation guidelines: Adequate hydration, IV access, physical examination, pediatric surgery consultation (in case of perforation)
* If child appears not well (lethargic), good idea to have surgery present at time of reduction
* Guidelines: Good rectal seal, 120 mmHg maximal pressure at rest but can be greater during crying/Valsalva, typically three attempts during any one sitting, after rest period additional attempts can be made
* Intussusception encountered as round mass that moves retrograde with increased pressure
* Success: Reflux of gas into small bowel, resolution of soft tissue mass
* Mass most likely to "get stuck" at ileocecal valve
* If initial progression of mass on initial attempts but not able to reduce beyond ileocecal valve, a period of an hour may allow for edema to decrease and increased chance of success
* May be difficult at times to differentiate edematous ileocecal valve from persistent intussusception: Follow clinically
o Edematous ileocecal valve may predispose to recurrence
* Success rates 80-90% with air reduction
* Risk of perforation 0.5%
* Recurrences can be treated on up to three occurrences prior to considering surgical exploration for potential pathological lead point
NEC
Terminology

* Idiopathic enterocolitis in very low birth weight premature infants most likely related to some combination of infection and ischemia characterized by coagulative and hemorrhagic necrosis and inflammation of portions of the small and large intestine

Imaging Findings

* Best diagnostic clue: Pneumatosis
* Best imaging tool: Radiography +/- sonography
* Suspected acute NEC: Serial abdominal radiographs
* Question ischemic bowel: Sonography with color Doppler

Pathology

* Multifactorial etiology
* Incidence: 0.3-2.4 cases per 1,000 live births
* Increased; more premature babies surviving

Clinical Issues

* Most common signs/symptoms: Abdominal distention, bloody stools, diarrhea, feeding intolerance, increased gastric aspirates, sepsis, apnea and bradycardia, lethargy, temperature instability
* 1-3 week old premature infant; often with (HMD)
* 10% are term babies
* Overall mortality 20-30%

Diagnostic Checklist

* Asymmetric bowel distention
* Pneumatosis may be linear or bubbly
* PVG closer to the liver capsule than biliary gas
Crohns
Terminology

* Terminal ileitis, regional enteritis, ileocolitis, granulomatous colitis, Crohn disease (CD)

Imaging Findings

* Barium/CT
* Segmental ileo-colonic ulceration & wall thickening
* Acute or noncicatrizing phase: Minimal luminal narrowing
* Chronic or cicatrizing phase: ↑ Luminal narrowing & no "target" sign

Top Differential Diagnoses

* Ulcerative colitis (UC)
* Appendicitis
* Mesenteric adenitis
* Infectious colitis

Pathology

* Transmural inflammation affecting all layers to the serosa
* Genetic, environmental, infectious, immunologic & psychologic factors
* Annual incidence: 0.6-6.3 cases/100,000 population
* Risk factors
* Smoking: Increased (4x); decreased risk in UC
* Caucasian race, Jewish (2-4x), oral contraceptive pills, & diet

Clinical Issues

* Recurrence: 30-53% after resection; 10-20% lead symptom free lives
* Fissures, sinus tracts, fistulas & abscesses: Hallmark (20-40%)
Germinal matrix bleed
Terminology

* Hemorrhage which occurs in the germinal zone, a specialized, highly vascular region in the ventricular walls where the neurons and glia are generated

Imaging Findings

* Best diagnostic clue: Echogenic area in the ventricular wall (usually caudothalamic notch) which may extend into the lateral ventricle(s) or periventricular brain parenchyma

Top Differential Diagnoses

* Porencephaly or Cystic Periventricular Leukomalacia
* Choroid Plexus Cysts or Hematoma
* Ventriculitis
* Ischemia or Infarction
* Intraventricular Hemorrhage

Pathology

* Most GMHs occur in the first week of life
* Related to perinatal stresses: Labile blood pressure, hypoxia, hypercarbia, etc.
* Most common in infants < 32 weeks gestation and < 1000 grams
* Higher risk in premies who have congenital heart disease, surgical procedures, severe respiratory distress
* Grade I: Confined to the caudothalamic groove
* Grade II: Extends into the ventricle but does not expand it
* Grade III: Fills and distends the adjacent ventricle
* Grade IV: Parenchymal hemorrhagic venous infarction (PVHI)
child abuse
* Most common cause of serious intracranial injuries in children less than 1 year of age
* 3rd most common cause of death in children after sudden infant death syndrome and true accidents
* Prevalence
o 1.7 million cases reported, 833,000 of which were substantiated in United States in 1990
o Results in 2,500-5,000 deaths/year
o 5-10% of children seen in emergency rooms suffer from child abuse
* Radiologist has legal obligation to report suspected child abuse, usually to the referring physician
* Age
o Usually <2 years
* In children <2 years of age, a skeletal survey may be best to demonstrate other fractures
o In children >2 years of age, a bone scan may be best
* Clinical findings
o Skin burns
o Bruises
o Lacerations
o Hematomas
* Skeletal trauma is seen in 50-80%





Skeletal Trauma Suspicious for Child Abuse



Site(s)


Remarks

Distal Femur, distal humerus, wrist, ankle


Metaphyseal corner fractures

Multiple


Fractures in different stages of healing

Femur, humerus, tibia


Spiral fractures < 1 year of age

Posterior ribs, avulsed spinous processes


Unusual “naturally-occurring” fractures <5years of age

Multiple skull fractures


Multiple fractures of occipital bone should suggest child abuse

Fractures with abundant callous formation


Implies repeated trauma and no immobilization

Metacarpal and metatarsal fractures


Unusual “naturally-occurring” fractures <5years of age

Sternal and scapular fractures

Vertebral body fractures and subluxations



* Sites of skeletal trauma
o Multiple ribs
o Transverse fracture of sternum
o Costochondral / costovertebral separation
o Lateral end of clavicles
o Scapula
o Acromion
o Skull
o Vertebral bodies
+ Anterior-superior wedging of vertebral bodies
+ Vertebral compression
+ Vertebral fracture dislocation
+ Disk space narrowing
+ Spinous processes

Frontal radiograph of the chest demonstrates multiple rib fractures with callous formation, including a fracture of the left 2nd and 6th ribs posteriorly. Posterior rib fractures are highly suggestive of child abuse (from forceful squeezing)


* Appearances of skeletal trauma
o Hallmark of the syndrome are multiple, asymmetric fractures in different stages of healing
o Separation of distal epiphysis
o Marked irregularity and fragmentation of metaphyses
+ "Corner" fracture (11%) or "Bucket-handle" fracture = avulsion of a metaphyseal fragment overlying the lucent epiphyseal cartilage secondary to a sudden twisting motion of extremity
o Isolated spiral fracture (15%) of diaphysis secondary to external rotatory force applied to femur / humerus
o Extensive periosteal reaction from large subperiosteal hematoma
o Exuberant callus formation at fracture sites
o Cortical hyperostosis extending to epiphyseal plate
o Avulsion fracture at site of ligamentous insertion
+ Frequently seen without periosteal reaction
* Head trauma (13-25%)
o Most common cause of death and/or physical disability
+ Skull fracture (flexible calvaria + meninges decrease likelihood of skull fractures)
+ Subdural hematoma
+ Brain contusion
+ Cerebral hemorrhage
+ Infarction
+ Generalized edema
+ Shearing injuries with associated subarachnoid hemorrhage
+ Skull film (associated fracture in 1%):
# Linear fracture > comminuted fracture
o CT findings in head trauma
+ Subdural hemorrhage (most common)
# Interhemispheric location most common
+ Subarachnoid hemorrhage
+ Epidural hemorrhage (uncommon)
+ Cerebral edema (focal, multifocal, diffuse)
+ Acute cerebral contusion appears as ovoid collection of intraparenchymal blood with surrounding edema
o MR findings of head trauma
+ More sensitive in identifying hematomas of differing ages
+ White matter shearing injuries as areas of prolonged T1 + T2 at corticomedullary junction, centrum semiovale, corpus callosum
* Viscera (3%)
o Second leading cause of death in child abuse
o Cause
+ Crushing blow to abdomen (punch, kick)
o Age
+ Often >2 years
o Small bowel and/or gastric rupture
o Hematoma of duodenum and/or jejunum
o Contusion and/or laceration of lung, pancreas, liver, spleen, kidney
o Traumatic pancreatic pseudocyst
* Differential diagnosis of child abuse
o Normal periostitis of infancy
o Osteogenesis imperfecta
o Congenital insensitivity to pain
o Infantile cortical hyperostosis
o Menkes kinky hair syndrome
o Schmid-type chondrometaphyseal dysplasia
o Scurvy
o Congenital syphilitic metaphysitis
Vesicoureteral Reflux
Terminology

* Retrograde flow of urine from the bladder toward the kidney

Imaging Findings

* Best diagnostic clue: Contrast instilled into the bladder opacifies the ureter and may reach the intrarenal collecting system, often only seen transiently
* VCUG preferred whenever anatomic detail of the upper tracts is needed and in all cases when urethral anatomy is being evaluated
* Nuclear cystogram preferred when anatomy is known and for follow-up studies
* Cystosonography performed in countries where ultrasound contrast agents are available

Pathology

* Shortened or abnormally angulated insertion of ureter into bladder is theorized to result in VUR
* The vast majority (80%) of pediatric patients outgrow VUR, presumably due to changes at the level of the ureterovesicle junction, often associated with a growth spurt
* Clear association with acute pyelonephritis
* International Reflux Study Committee grading system of vesicoureteral reflux
* I: Reflux into ureter not reaching the renal pelvis
* II: Reflux reaching pelvis but no blunting of calyces
* III: Mild caliceal blunting
* IV: Progressive caliceal and ureteral dilation
* V: Very dilated and tortuous collecting system, intrarenal reflux
Epiglottis and DDX
Terminology

* Airway obstruction secondary to infectious inflammation of the epiglottis and surrounding tissues

Imaging Findings

* Classic imaging appearance: Lateral radiograph shows enlargement of epiglottis and thickening of the aryepiglottic folds
* Not to be confused with "omega" epiglottis: Normal variant when epiglottis imaged obliquely

Pathology

* Typically older than patients with croup
* Since vaccine for Haemophilus influenzae (Hib) is available, incidence of epiglottitis has markedly decreased
* More cases of epiglottitis resulting from other bacterial organisms, viral or combined viral-bacterial infections are now seen since introduction of Hib vaccination
* With introduction of Hib vaccine, epidemiology has shifted toward significantly older patients
* Epiglottitis may be seen in older patients and even those previously immunized against Hib

Clinical Issues

* Increased respiratory distress when recumbent (reason why radiographs obtained upright or whatever way patient comfortable)
* Life threatening disease often requiring emergent intubation

Diff:

Omega Epiglottis (Normal Variant)

* If epiglottis obliquely imaged, can appear artificially wide because left and right sides of epiglottis are being imaged adjacent to each other causing an "omega" shaped appearance
* Thickening of aryepiglottic folds is absent

Aspirated Bronchial Foreign Body

* Radiopaque foreign body seen in minority of cases
* Asymmetric lung aeration on chest radiographs

Croup

* Benign, self-limited condition
* Most common acute airway condition encountered
* Symmetric subglottic narrowing

Exudative Tracheitis

* Children typically older than those with croup
* Intraluminal filling defect (membrane), tracheal wall plaque-like irregularity, asymmetric subglottic narrowing

Retropharyngeal Abscess (RPA)

* Pyogenic infection of retropharyngeal space
Croup and DDX
Terminology

* Acute laryngotracheobronchitis
* Self-limited viral inflammation of the airways resulting in symmetric subglottic edema and croupy cough

Imaging Findings

* Loss of normal shoulders (lateral convexities) of the subglottic trachea secondary to subglottic edema: "Steeple sign" or inverted "V"
* Symmetric, subglottic narrowing with narrow portion of airway extending more inferiorly than level of the pyriform sinuses on frontal view

Top Differential Diagnoses

* Aspirated Bronchial Foreign Body
* Epiglottitis
* Exudative Tracheitis

Pathology

* Benign, self-limited condition secondary to viral illness
* Most common cause of upper airway obstruction in young children

Clinical Issues

* Most common signs/symptoms: Acute clinical syndrome characterized by barky ("croupy") cough, inspiratory stridor, and hoarseness
* Peak age: 1 year
* Managed supportively as outpatients
* Oral or inhaled corticosteroids have become more routinely used as therapy for all children with croup

Diff:
Aspirated Bronchial Foreign Body

* Radiopaque foreign body seen in minority of cases
* Most common foreign body in main bronchi
* Tracheal foreign bodies rare
* Asymmetric lung aeration on chest radiographs

Epiglottitis

* Typically older than croup: Mean age of epiglottitis = 3 years
* Severe, life-threatening condition
* Marked enlargement of epiglottis and aryepiglottic folds
* May cause symmetric subglottic narrowing on frontal view: Similar in appearance to croup

Exudative Tracheitis

* Children typically older than those with croup
* Intraluminal filling defect (membrane)
* Tracheal wall plaque-like irregularity
* Asymmetric subglottic narrowing
Croup and DDX
Terminology

* Acute laryngotracheobronchitis
* Self-limited viral inflammation of the airways resulting in symmetric subglottic edema and croupy cough

Imaging Findings

* Loss of normal shoulders (lateral convexities) of the subglottic trachea secondary to subglottic edema: "Steeple sign" or inverted "V"
* Symmetric, subglottic narrowing with narrow portion of airway extending more inferiorly than level of the pyriform sinuses on frontal view

Top Differential Diagnoses

* Aspirated Bronchial Foreign Body
* Epiglottitis
* Exudative Tracheitis

Pathology

* Benign, self-limited condition secondary to viral illness
* Most common cause of upper airway obstruction in young children

Clinical Issues

* Most common signs/symptoms: Acute clinical syndrome characterized by barky ("croupy") cough, inspiratory stridor, and hoarseness
* Peak age: 1 year
* Managed supportively as outpatients
* Oral or inhaled corticosteroids have become more routinely used as therapy for all children with croup
Laryngeal papillomatosis
Terminology

* Laryngeal nodules due to human papilloma virus, usually self-limited infection
* < 1% seed the lung, known as invasive papillomatosis
* At risk to develop squamous cell carcinoma (2%)

Imaging Findings

* Best diagnostic clue: Multiple solid and cystic nodules
* Dorsal distribution in axial plane (gravity seeding)

Top Differential Diagnoses

* Metastases
* Wegener Granulomatosis
* Pneumatoceles
* Lymphangioleiomyomatosis

Pathology

* Virus also responsible for cutaneous warts, genital warts and cervical cancer

Clinical Issues

* Hoarseness most common due to laryngeal involvement
* Lung nodules grow very slowly, usually measured in decades

Diagnostic Checklist

* Nodules grow slowly, and sudden growth or change in nodule appearance must be investigated for transformation to squamous cell carcinoma
* Always evaluate the trachea for nodules in young patient with multiple cavities
Polyposis Syndromes
Abbreviations and Synonyms

* Peutz-Jeghers syndrome (PJS)
* Multiple hamartoma syndrome/Cowden disease (MHS)
* Juvenile polyposis (JP)
* Cronkhite-Canada syndrome (CCS)

Definitions

* Spectrum of both hereditary & nonhereditary polyposis syndromes characterized by gastrointestinal tract (GIT) polyps & other associated lesions
Terminology

* Spectrum of both hereditary & nonhereditary polyposis syndromes characterized by gastrointestinal tract (GIT) polyps & other associated lesions

Imaging Findings

* Best diagnostic clue: Cluster of small radiolucent filling defects in small bowel (PJS)
* Jejunum + ileum > duodenum > colon > stomach
* PJS polyps occur in clusters > carpeting bowel

Top Differential Diagnoses

* Familial adenomatous polyposis
* Brunner gland hyperplasia (hamartoma)
* Lymphoid follicles (hyperplasia)
* Metastases & lymphoma (GI tract)

Pathology

* Hereditary (AD): PJS, MHS, 25% of JP
TTN
Terminology

* Transient tachypnea of the newborn (TTN), wet lung disease, retained fetal fluid
* Transient tachypnea occurs when liquid in the fetal lung is removed slowly or incompletely from newborn lung and there is increased absorption by lymphatics and capillaries

Imaging Findings

* Findings similar to pulmonary edema
* Normal heart size
* Prominent intersitial markings
* Diffuse, bilateral and commonly symmetric increased lung markings which becomes normal within 24-48 hours
* ± Pleural effusion
* ± Fluid in the fissures

Top Differential Diagnoses

* Congenital Heart Disease (CHD)
* Meconium Aspiration Syndrome
* Congenital Lymphangiectasia
* Neonatal Pneumonia

Pathology

* Incidence is 11 per 1,000 live births
* Not associated with any mortality or morbidity

Clinical Issues

* Newborns
* Initial mild to moderate respiratory distress at birth or within six hours
* Relatively benign clinical course
* Exclude other causes of tachypnea in a term newborn
Meconium Asp.
Terminology

* Respiratory distress that occurs as the result of the aspiration of meconium

Imaging Findings

* Best diagnostic clue: Coarse bilateral lung opacities with increased lung volumes in a term infant
* Pleural effusion uncommon
* Best imaging tool: Chest radiograph

Top Differential Diagnoses

* Congenital Heart Disease (CHD)
* Neonatal Pneumonia
* Transient Tachypnea of the Newborn (TTN)
* Congenital Chest Mass such as Congenital Cystic Adenomatoid Malformation (CCAM)

Pathology

* Meconium is a tenacious, thick and viscous material in neonatal bowel
* Occurs in term infants who have in-utero or intrapartum hypoxia or stress
* Meconium may be detected in amniotic fluid of 10-20% of newborns > 34 weeks gestation
* 25,000-30,000 cases of meconium aspiration yearly in USA

Clinical Issues

* Cyanosis, nasal flaring, intercostal retractions
* Airway obstruction
* Surfactant dysfunction
* Chemical pneumonitis
* Meconium injury contributes to high pulmonary vascular resistance
Neonatal pneumonia
Terminology

* Pneumonia occurring in neonate within the first 28 days of life
* Lung infection occurs in utero, during delivery, or within the first month of life

Imaging Findings

* Group B pneumonia
* Most common neonatal pneumonia
* Different appearance than other causes of neonatal pneumonia
* Low lung volumes and granular opacities similar to surfactant deficiency
* Pleural effusion in 25%: Only differentiating factor from surfactant deficiency
* Other types of neonatal pneumonia
* Bilateral hyperinflation
* Rope-like perihilar markings
* Best imaging tool: Chest radiography

Top Differential Diagnoses

* Surfactant Deficient Disease
* Congenital Cystic Adenomatoid Malformation (CCAM) of the Chest
* Meconium Aspiration Syndrome
* Transient Tachypnea of the Newborn
* Congenital Heart Disease

Pathology

* Occurs in approximately 1% of term neonates and 10% of preterm neonates

Diagnostic Checklist

* Usually bilateral diffuse disease
* Chest radiographs are nonspecific
Ewings - especially of the scapula
Terminology

* Round cell sarcoma of bone

Imaging Findings

* Centered in vertebral body or sacrum
* Permeative/moth eaten bone destruction
* Wide zone of transition
* Tiny perforations of cortex rather than extensive loss of cortical bone radiographically
* 50% have extraosseous, noncalcified, soft tissue mass
* MR best shows involvement of adjacent bones & soft tissues

Top Differential Diagnoses

* Primitive Neuroectodermal Tumor (PNET)
* Langerhans Cell Histiocytosis
* Other Small Round Cell Tumors
* Osteomyelitis
* Osteogenic Sarcoma (OGS)
* Other Primary Sarcomas and Metastatic Disease

Pathology

* Primitive neuroectodermal tumor (PNET) is closely related tumor
* 6th most common malignant bone tumor

Clinical Issues

* Localized pain
* Fever, leukocytosis, elevated ESR (simulating osteomyelitis)
* Prognosis worse in spinal than peripheral Ewing sarcoma because of difficulty of surgical resection
* Significant risk of second malignancy
Abdominal mass in older child:
renal – Wilms, hydronephrosis; GI – appendiceal abscess, intussusception, neoplasm; RP – neuroblastoma; genital – ovarian cyst, teratoma, hydrometrocolpos; hepatobiliary – hepatoblastoma, HCC, mesenchymal hamartoma; VP shunt – CSFoma
Recurrent pneumonia
sequestration
Foreign body
TE fistula and types
sequestration
Terminology

* Congenital area of abnormal lung that does not connect to the bronchial tree or pulmonary arteries
* Involved lung is dysplastic and nonfunctioning
* Arterial supply is typically from systemic source arising from descending aorta
* Divided into intralobar and extralobar types
* Intralobar type has venous drainage into inferior pulmonary vein
* Extralobar type has venous drainage often systemic, however drainage variable
* May occur in conjunction with other congenital lung lesions such as congenital cystic adenomatoid malformation

Imaging Findings

* Persistent lung opacity over multiple presentations with pneumonia-like symptoms
* Most common location is left lower lobe, followed by right lower lobe
* Systemic arterial supply most commonly arises from descending aorta
* May arise from below the hemidiaphragm in 20% of cases
* Diagnostic feature: Systemic artery arising from the aorta and feeding sequestration
* Identification of the supplying systemic artery is characteristic and documentation that surgeons are interested in
Foreign body
Imaging Findings

* Static lung volume at different phases of respiratory cycle (need more than inspiratory chest radiograph alone)
* Alternatives include inspiratory/expiratory radiographs in cooperative patients, fluoroscopy, bilateral decubitus radiographs, radiographs at forced expiration
* Bronchial foreign bodies more common than elsewhere
* Bronchial 76%, laryngeal 6%, tracheal 4%
* Volume of affected lung segments can be normal, increased, or decreased
* Reported statistics on presence of findings on static chest radiograph
* Normal: 14-35%
* Obstructive emphysema: 21-43%
* Opacification/atelectasis: 18-29%
* Mediastinal shift: 36.8%
* Radiopaque foreign body: 3-23%

Clinical Issues

* Symptoms may be indolent: Occult presentation (10-25%)
* Typically present with wheezing, cough, sometimes fever
* Typically 8 months to 3 years of age
* 57% between 1 and 2 years of age
* Delay in diagnosis associated with increased risk of major complications
TE fistula and types
Terminology

* Faulty division of the foregut into tracheal and esophageal channels during the first month of embryogenesis

Imaging Findings

* Best diagnostic clue: Distended pharyngeal pouch with tip of enteric tube within it
* Best imaging tool: Radiography for initial diagnosis +/- air in proximal pouch by fluoroscopy
* Upper GI for isolated (H-type) TEF
* Lateral position for H-type

Pathology

* Faulty division of the foregut into tracheal and esophageal channels, first month gestation
* 50-75% of those have associated abnormalities
* EA with distal TEF: 82%
* EA with no TEF: 9%
* Isolated (H-type) TEF: 6%
* EA with proximal and distal TEF: 2%
* EA with proximal TEF: 1%

Clinical Issues

* Most common signs/symptoms: Excessive oral and pharyngeal secretions or choking, cyanosis, or coughing during first attempt at feeding
* H-type fistula
* Coughing or choking during feeding
* Recurrent pneumonia
* Postsurgical survival: 75-95% (dependent on associated cardiac defects)
* Surgical
Posterior Urethral valves

Oblique voiding cystourethrogram shows dilated posterior urethra and thin valve tissue extending from the ventral surface of the urethra (arrow). The urethral caliber narrows just distal to the valve tissue.
Terminology

* Varying degree of chronic urethral obstruction due to fusion and prominence of plicae colliculi, normal concentric folds of urethra
* Classic imaging appearance: Abrupt transition on VCUG from dilated posterior urethra to small bulbous urethra at the level of the valves, actual valve tissue may not be visible
* Occurs exclusively in males

Imaging Findings

* VCUG is the gold standard for imaging posterior urethral valves
* Associated findings
* Bladder wall trabeculation/muscular hypertrophy
* Vesicoureteral reflux
* Urinary ascites
* Urinoma/perinephric urine collection
* Reflux into utricle or other ducts
* MR is superior to ultrasound in older fetuses whose ossifying pelvic bones obscure visualization of the bladder outlet

Top Differential Diagnoses

* Anterior urethral valves
* Voiding dysfunction
* Cecoureterocele
* Post-surgical or post-traumatic urethral stricture

Pathology

* Incidence between 1 in 8,000 to 25,000 births
* 80% have associated vesicoureteral reflux
Appy - plain film and US
Terminology

* Acute obstruction of the appendiceal lumen results in distention of the appendix, superimposed infection, ischemia, and eventually perforation

Imaging Findings

* Best diagnostic clue: Appendicolith
* Location: Normally right lower quadrant (RLQ), but can be in left lower quadrant with malrotation, rarely
* Morphology: Dilated enhancing tubular structure in RLQ with associated mesoappendix soft tissue stranding
* Noncompressible blind-ending tubular structure over 6 mm in diameter
* Much debate concerning imaging algorithms for appendicitis

Top Differential Diagnoses

* Mesenteric adenitis
* Girls: Right ovarian pathology

Pathology

* Most common reason for abdominal surgery in children
* 15-20% of specimens may be negative

Clinical Issues

* Classic symptoms in older children with nonperforated appendicitis
* Pain begins in periumbilical region
* Migration to right lower quadrant
* Tenderness over McBurney's point
Renal mass DDx
Cystic renal masses: hydronephrosis, ARPKD, MCDK, multilocular cystic nephroma, VHL, TS, cystic Wilms

Solid renal mass: Wilms (children), mesoblastic nephroma (newborns), nephroblastomatosis, AML, lymphoma, leukemia, neuroblastoma
Renal mass
Top Differential Diagnoses

* Wilms tumor
* Neuroblastoma
* Ganglioneuroma
* Adrenal hemorrhage
* Autosomal recessive polycystic kidney disease
* Multicystic dysplastic kidney
* Extrapulmonary sequestration
* Ossifying renal tumor of infancy
Adrenal mass DDx
neonatal hemorrhage (common), neuroblastoma, other RP masses (Wilms, hydronephrotic upper pole,
ruptured spleen
Terminology

* Parenchymal injury to spleen with or without capsular disruption

Imaging Findings

* Low attenuation laceration or subcapsular hematoma
* Surrounding perisplenic hematoma [> 30 Hounsfield units (HU)]
* Classic imaging appearance
* Low density intraparenchymal hematoma
* Subcapsular hematoma flattens or compresses outer splenic margin
* Active arterial extravasation diagnosed when high attenuation focus isodense to aorta is seen within an area of low density hematoma
* CT accuracy: 98%; sensitivity: 95%

Top Differential Diagnoses

* Bolus artifact
* Splenic cleft
* Splenic infarct
* Splenic abscess

Pathology

* Etiology: Blunt trauma; (MVA #1 & handle-bar injuries)

Diagnostic Checklist

* Bolus artifact or congenital cleft in patients lacking perisplenic hematoma
panc. laceration
Imaging Findings

* Best diagnostic clue: Enlargement of gland, heterogeneous parenchyma, peripancreatic fluid collections, history of trauma
* Morphology: Spectrum of injury: Acute pancreatitis, contusions, deep lacerations, fractures with ductal disruption
* Irregularity of pancreatic contour
* Edema/fluid in peripancreatic fat
* Rupture of main pancreatic duct (MPD) (23%)
* Protocol advice: 24-48 hours delayed scans may uncover findings not present earlier

Top Differential Diagnoses

* "Shock" Pancreas
* Intramural Duodenal Hematoma ± Duodenal Rupture
* Pancreatitis

Pathology

* Penetrating/blunt trauma
* Pancreatic trauma uncommon, 3-12% of all abdominal injuries

Clinical Issues

* Complications: Recurrent pancreatitis, pseudocyst, hemorrhage, pseudoaneurysm, fistula, abscess
* Mortality from pancreatic injuries is nearly 20%

Diagnostic Checklist

* CT diagnosis of pancreatic trauma may be difficult in patients scanned soon after injury
* CT signs of traumatic pancreatitis become more evident after 24-48 hours
Lateral upper GI (same child) shows a partially obstructing rounded intraluminal mass (open arrow) in 2nd portion of duodenum caused by a large duodenal hematoma.
Imaging Findings

* CECT: Mass 2nd or 3rd portion of duodenum in child with trauma, esophagogastroduodenoscopy (EGD), or bleeding disorder
* Upper gastrointestinal (UGI): Intraluminal filling defect or obstruction of duodenum
* Most common site of bowel injury in blunt abdominal trauma (25%)
* Important to assess for duodenal perforation (requires emergency surgical repair)
* Frequently associated with pancreatic injury in cases of blunt trauma

Top Differential Diagnoses

* Enteric duplication cyst
* Small bowel malrotation/midgut volvulus

Clinical Issues

* Vomiting
* Abdominal pain
* Excellent prognosis
* Supportive care
* Resolves spontaneously
* Surgical repair
* If conservative management fails

Diagnostic Checklist

* Duodenal hematoma in vomiting child with
* Blunt abdominal trauma (most common)
* EGD with biopsy
* Bleeding disorder
* HSP
Opacified Hemithorax
Opacified Hemithorax

1. Atelectasis

2. Effusion

3. Pneumonia

4. Post-pneumonectomy
Elevated hemidiaphragm DDx
* above the diaphragm - decreased lung volume
o atelectasis / collapse
o lobectomy / pneumonectomy
o pulmonary hypoplasia
* diaphragm
o phrenic nerve palsy
o diaphragmatic eventration
* below the diaphragm
o abdominal tumour
o subphrenic abscess
o distended stomach or colon.
Rhabdomyosarcoma, Genitourinary
Terminology

* Rhabdomyosarcoma originating from any of the pelvic organs

Imaging Findings

* Tumors may be cystic or solid, are typically large and cause significant mass effect
* Secondary urinary obstruction is common
* Tumors spread by local extension, via lymphatics, and hematogenous metastases to lungs, liver, and bone

Top Differential Diagnoses

* Complex ureterocele
* Bladder hematoma
* Ovarian tumor
* Pelvic neuroblastoma
* Pelvic lymphoma/Burkitt
* Hematometrocolpos
* Sacrococcygeal teratoma
* Pelvic inflammatory disease/abscess

Pathology

* Four major histologic types
* Embryonal cell type, accounts for 55%
* Botryoid variant of embryonal type, 5%
* Alveolar rhabdomyosarcoma, 20%
* Undifferentiated type, 20%

Clinical Issues

* Peak incidence 2-6 years old
* Surgery, chemotherapy, and radiation therapy combined
Anteroposterior radiograph during percutaneous cholangiogram shows fusiform dilation of the common bile duct with rapid change in caliber at the sphincter of Oddi, confirming a type I choledochal cyst.
Terminology

* Choledochal malformations, common bile duct cyst or diverticulum, choledochocele
* Choledochal cysts are a spectrum of malformations of the extrahepatic and intrahepatic bile ducts

Imaging Findings

* Ultrasound is best initial screening test
* Other cross sectional imaging (CT, MRI) for additional anatomic detail

Top Differential Diagnoses

* Chronic cholangitis
* Obstructing cholelithiasis
* Pancreatic pseudocyst
* Hydatid cyst

Pathology

* Type I cysts are characterized by segmental or diffuse fusiform dilatation of the common bile duct; they are the most common variety, accounting for 75-95% of cases
* Type II cysts represent a diverticulum of the duct, usually protruding from the lateral wall
* Type III cysts represent a choledochocele that most often occurs within the duodenal wall and protrudes as mass into duodenal lumen
* Type IV designates the presence of multiple extrahepatic bile duct cysts; this can be seen alone (type IV B) or in association with Caroli-type intrahepatic biliary cysts (type IV A)
* Type V is cystic dilatation of the intrahepatic bile ducts equivalent to Caroli disease
Caudal regression
Terminology

* Caudal regression syndrome (CRS), sacral agenesis, lumbosacral dysgenesis

Imaging Findings

* Best diagnostic clue: Dysgenetic lumbosacral vertebrae with abnormal distal spinal cord
* Spectrum ranging in severity from absent coccyx to complete lumbosacral agenesis
* Group 1: Blunt spinal cord termination above L1; central canal may be prominent
* Group 2: Elongated conus with thick filum +/- intraspinal lipoma

Top Differential Diagnoses

* Tethered Spinal Cord
* Closed Spinal Dysraphism
* Occult Intrasacral Meningocele (OIM)

Pathology

* Most severe cases ⇒ sirenomelia ("mermaid")
* Most cases sporadic
* 1/7,500 births (milder forms > severe forms)
* 15-20% are infants of diabetic mothers; 1% of offspring from diabetic mothers affected
* Genitourinary abnormalities (24%)

Diagnostic Checklist

* MR imaging most useful imaging tool for characterizing abnormalities and surgical planning
* Caudal spine anomalies should be sought out in patients with genitourinary or anorectal anomalies and vice versa
Prune Belly Syndrome
Terminology

* Rare congenital disorder characterized by dramatic collecting system dilatation, deficiency of abdominal musculature and cryptorchidism

Imaging Findings

* Large, thin-walled bladder
* Bilateral hydroureter
* Bilateral hydronephrosis
* Entire urethra dilated
* Need to focus scan on urethra
* Scan genitalia, looking for undescended testes

Top Differential Diagnoses

* Posterior urethral valves
* Megacystis-microcolon

Pathology

* Large bladder inhibits descent of testes
* Rarely reported in females
Lymphoid hyperplasia on BE
Terminology

* Intussusception: Forward peristalsis results in invagination of more proximal bowel (the intussusceptum) into lumen of more distal bowel (the intussuscipiens) in a telescope-like manner

Imaging Findings

* Best diagnostic clue: Radiography: Meniscus of soft tissue mass outlined in air-filled colon
* Most common site: Terminal ileum/ileocecal valve

Pathology

* Bowel wall congestion from venous obstruction may lead to bowel ischemia and necrosis
* Seasonal occurrence (winter, spring) with viral illnesses

Clinical Issues

* Alternating lethargy and irritability
* Colic
* Most common between 3 months-1 year of age
* Surgery reserved for cases of reduction failure
* Success rates 80-90% with air reduction
* Risk of perforation 0.5%

Diagnostic Checklist

* Left-side-down decubitus/prone views can be helpful in showing lack of air-filled cecum
* CT: Colonic mass with alternating rings of high and low attenuation
* May not be located in RLQ if intussusception has progressed distally
Varicella pneumonia
* Varicella-zoster virus pneumonia is the most serious complication of disseminated varicella-zoster virus infection with mortality rates of 9%–50%.
* Varicella-zoster virus most commonly causes self-limited benign disease (chickenpox) in children. However, in adults it tends to cause significant complications such as varicella-zoster virus pneumonia.
* More than 90% of cases of varicella-zoster virus pneumonia in adults occur in patients with lymphoma and immunocompromised patients.
* Histologic features of varicella-zoster virus pneumonia are those of diffuse alveolar damage. With recovery from the initial disease, spherical nodules are seen, scattered randomly throughout the lung parenchyma.

[edit] Imaging Findings
[edit] Plain film

* Multiple 5–10-mm ill-defined nodules that may be confluent and fleeting.
* Small, round nodules usually resolve within a week after the disappearance of the skin lesions but may persist for months.
* Lesions can calcify and can persist as numerous, well-defined, randomly scattered, 2–3-mm dense calcifications.

[edit] CT

* Well-defined and ill-defined nodules diffusely throughout both lungs.
* Nodules with a surrounding halo of ground-glass opacity, patchy ground-glass opacity, and coalescence of nodules are also seen.
Varicella pneumonia
* Varicella-zoster virus pneumonia is the most serious complication of disseminated varicella-zoster virus infection with mortality rates of 9%–50%.
* Varicella-zoster virus most commonly causes self-limited benign disease (chickenpox) in children. However, in adults it tends to cause significant complications such as varicella-zoster virus pneumonia.
* More than 90% of cases of varicella-zoster virus pneumonia in adults occur in patients with lymphoma and immunocompromised patients.
* Histologic features of varicella-zoster virus pneumonia are those of diffuse alveolar damage. With recovery from the initial disease, spherical nodules are seen, scattered randomly throughout the lung parenchyma.

[edit] Imaging Findings
[edit] Plain film

* Multiple 5–10-mm ill-defined nodules that may be confluent and fleeting.
* Small, round nodules usually resolve within a week after the disappearance of the skin lesions but may persist for months.
* Lesions can calcify and can persist as numerous, well-defined, randomly scattered, 2–3-mm dense calcifications.

[edit] CT

* Well-defined and ill-defined nodules diffusely throughout both lungs.
* Nodules with a surrounding halo of ground-glass opacity, patchy ground-glass opacity, and coalescence of nodules are also seen.
Cystic Fibrosis, Lung Posteroanterior radiograph shows coarse reticulonodular (white arrows) opacities of peribronchial thickening and mucous plugging. Also note exacerbating lingular consolidation (black arrow).
Terminology

* Autosomal recessive multisystem disorder of exocrine gland function characterized in the respiratory tract by recurrent infection, chronic obstruction and chronic sinusitis with nasal polyps

Imaging Findings

* Best diagnostic clue: Predominately upper lobe bronchiectasis and mucous plugging in hyperinflated lungs
* Predisposed to spontaneous pneumothorax
* "Signet ring sign" on CT: Dilated bronchus in association with adjacent artery on axial images
* Secretions within peripheral small centrilobular bronchioles can give V- or Y-shaped opacities: "Tree-in-bud"
* HRCT will detect subtle disease and will better demonstrate extent of changes of CF and complications
* Consider controlled ventilation technique for HRCT in infants and young children

Top Differential Diagnoses

* Recurrent aspiration
* Asthma
* Bronchopulmonary dysplasia
* Immotile cilia syndrome

Clinical Issues

* Highest prevalence in Caucasians of Northern European origin: 1:3,200
* Most fatalities due to progressive lung disease but also liver failure and massive hemoptysis
Juvenile Nasopharyngeal Angiofibroma
Terminology

* Benign but aggressive hypervascular mass arising from sphenopalatine foramen (SPF) on lateral nasopharyngeal wall

Imaging Findings

* Extends laterally into pterygopalatine fossa (PPF, pterygomaxillary fossa) in 90% of cases
* Obliteration of normal fat attenuation in PPF
* Enlarged ECA branches supply tumor that has dense capillary blush and delayed wash out
* Distal branches of internal maxillary artery invariably involved
* MR with contrast and fat-saturation essential for identifying intracranial invasion
* Multiplanar reformatting and volume rendering can help in surgical planning

Top Differential Diagnoses

* Hypervascular Polyp
* Rhabdomyosarcoma

Pathology

* Pathologic characteristics indicate it may be a vascular malformation rather than a tumor

Clinical Issues

* Nasal stuffiness: 90%
* Epistaxis: 60%
* Nearly exclusive male incidence ⇒ 75% have androgen receptors
* Local recurrence after surgery in up to 25%
* Surgical resection with pre-operative embolization
* Particles or liquid embolics to reduce blood loss
* Surgery in 24-72 hours
Septic Hip and evaluation
# Septic Joint

* Focal osteopenia; large effusion
* Requires aspiration for confirmation
* Transient synovitis (ages 3-10) mimics septic joint but noninfectious, a diagnosis of exclusion
Bowed bones: ddx
Anteroposterior radiograph shows bowed femurs and tibias with mild bowlegs in a 6.5 year old with hypophosphatemic rickets.

Bowed bones: rickets (most common), NF, OI, FD