Paediatrics Flashcards
Monoventricle, thalami and basal ganglia are fused?
Alobar Holoprosencephaly
Addition: Most severe form.
**No falx or corpus callosum
Complete absence of cleavage with “pancake” of anterior cerebral tissue, crescent-shaped anterior monoventricle communicating with large dorsal cyst,** fused thalami**
Multilocular fluid filled mass with internal septations at the the posterior triangle?
Cystic hygroma
Nb - Associated with turners and hydrops/downs
T2 bright. Doesnt enhance. No internal flow.
Difference and similarities between Epidermoid cyst and arachnoid cyst
Both - CSF density on CT, can occupy CPA, homogeneous.
Epidermoid - Restricts diffusion. Hyperintensity on FLAIR
Complete opacification of the maxillary sinus, peripheral enhancemen, expanded sinus with smooth remodeling of walls?
Mucocoele
Nb - Ostium of given sinus is occluded thus complete opacification.
*- Doesn’t extend beyond the cavity. *
- **Expands cavity and can cause bone thinning. **
Associated with* CF and prior trauma*
Peripheral enhancment allows differentiate from neoplasm
Low attenuation mass in the sinus that expands the cavity and extends into the meatus ?
Antrochonal polyp/Solitary Sinonasal Polyp
*Nb - T2 bright and also peripheral enhancement. *
Typically doesn’t completely opacify the sinus
Solitary ‘dumbell shaped-‘ polypoid mass fills maxillary antrum, then spills through enlarged maxillary ostium and infundibulum or accessory ostium into nasal cavity
Seizures, atrophy of hippocampus and high T2/FLAIR signal?
Mesial temporal sclerosis
Intraventricular lobulated mass in trigone of lateral ventricle. Smooth, large and small foci of calcification on CT. Lateral ventricle is dilated?
Choroid plexus papilloma
Hyperdense on CT.
Iso on T1 and hyperintense on T2.
**Intense homogenous enhancement. **
Nb in adults - occur predominantly in 4th ventricle
Mass in the inferior 4th ventricle of child with hypointensity on T1 and foci of high T2 signal ?
Ependymoma
4th ventricle mass (± indistinct interface with floor of 4th ventricle)
Fine, stippled Ca⁺⁺ common (50%)
± cysts, hemorrhage
Hydrocephalus common
Hetergenous Signal as Ca2+/Blood products
- overall T1 iso/hypointense and T2 hyerpintense
Anterolateral extension through recess(es) into CPA cistern
Posteroinferior extension through foramen of Magendie into cisterna magna
Erosive soft tissue mass in the middle ear ?
Cholesteatoma
Nb - its termed ‘Congenital’ if less than < 5yrs.
Isointense T1 and hyperintense T2
Resticts diffusion
Congenital cholesteatoma are less destructive.
Non- erosive soft tissue mass in the middle ear ?
Choleterol granuloma
Nb - Cystic strucutres with Blood and cholesterol =** High T1 and T2.**
If located in petrous apex can be aggressive
What brain tumour is found in Tuberous Sclerosis?
Subependymal giant cell astrocytoma (SEGA)
aka Intraventricular astrocytoma of tuberous sclerosis complex (TSC
Enlarging, enhancing foramen of Monro mass in patient with TSC
Tuberous sclerosis is triad of
-facial angiofibromas
-seizures
-mental retardation
Cortical/subcortical TUBERS
- expand overlying gyri, low on CT,
- can have cystic and Ca2+ transformation.
DDX - TORCH namely toxo and CMV can cause periventricular Ca2+
Highly vascular mass in nasopharynx eroding the medial ptyergoid plates and arising from sphenopalatine fossa?
Juvenile angiofibroma
Supplied by the internal maxillary artery. Embolise it.
Pineal mass in boy with precocious puberty?
Germinoma
Pineal mass with Central localized Ca⁺⁺ & bithalamic extension
Nb Germinoma and Pineoblastoma difficult to differentiate on imaging.
Can be Suprasellar mass with diabetes insipidus (DI)
Child with large, heterogeneous hyperdense pineal mass with peripheral Ca⁺⁺
Pineoblastoma
**Scattered (“exploded”) Ca⁺⁺ rather **than localized (“engulfed”) Ca⁺⁺ in germinoma
Differentiate a medulloblastoma from a pilocytic astrocytoma in the posterior cranial fossa
Medulloblastoma
Midline 4th ventricular mass in 1st decade of life
Hyperattenuating (CT)
diffusion restricting (MR)
PCA -
Cystic cerebellar mass with enhancing mural nodule
Enlarged optic nerve/chiasm/tract with variable enhancement
Hypo/Isodense. Ca2+ often
Distinguishing features if ADEM?
Post viral. Young adults or children
Demyelination with bilateral asymmetrical T2/FLAIR hyperintensities (confluent or punctuate).
Thalamic involvement and cranial nerves (not typcial MS)
Dramatic response with steorids
Chondrocalcinosis, premature OA changes and dark pigmentation in skin, particularly the ears?
Alkaptonuria / oschronosis
Nb Can be diagnosed in infancy - nappies stained black from urine.
AR condition. Excessive levels of homogentisic acid (HGA), affinity to accumulate in connective tissues.
Calcification and destruction intervertebral discs.
History of epilepsy, young, temporal lobe lesion, multi-cystic lesion?
Dysembryoplastic neuroepithelia tumours (DNET)
**Nb - Multiple cysts in the lesion ‘bubbly’ **.
Lack of oedema or enhancement is typical
DDX for a temporal lobe lesion and epilepsy -
—Ganglioglioma (not bubbly, can be cystic and usually enhance)
Baby, developmental delay, normal head size but diffuse white matter abnormalities with a stripey ‘trigoid’ appearance
Metachromatic Leukodystrophy
**Normal head size **
Most common leukodystrophy. Trigoid or butterfly appearance.
Spares subcortical u fibres
Baby under 6 months old, RUQ mass, cardiomegaly ?
Haemangioendothelioma (aka Infantile hepatic haemangiomas (IHH))
**Fine calcifications. **
Often found on foetal antenatal scans!!!
Can be solitary or multiple
Nb - behave like giant haemangioma on imaging -
Heterogenous on US, peripheral enhancement with gradual filling in on CT, flow voids on MRI.
can cause significant R-L heart shunting.
AFP + Endothelial growth factor is elevated.
Thrombocytopenia due to platelet trapping
Nb - not like adult epitheliod haemangioendothelioma
Painless abdominal mass, 6 year old child. US - large well defined solid heterogenous liver mass with raised AFP?
Hepatoblastoma
Coarse calcification
Precocious puberty is highly specific but rare.
Most common malignant hepatic tumour of childhood.
Young adolescents = HCC
Associated with* Beckwith Wiedemann syndrome. *
Can invade the vessels
Large abdominal mass in neonate, with respiratory compromise. Multicystic and enhancing septations.
Mesenchymal hamartoma
Benign tumour. Sheer size can cause diaphragms splinting.
No Ca2+ or haemmorhage.
May grow rapidly, leading to compartment syndrome, respiratory distress, rarely heart failure
AFP may be moderately elevated
Imaging: Multiloculated large cystic mass in 70%;
Neonatal jaundice, normal US and 6 and 24 hours HIDA scan show no excretion?
Biliary atresia
US - sometimes shows echogenic ‘triangular cord’ structure in the porta hepatis which is pathogonomic
DDx - Neonatal hepatitis - 6 or 24 hours HIDA will show bowel uptake
Nb
Hepatic activity after 5 minutes and no bowel activity after 6 and 24 hours
Main causes for Neonatal distal intestinal obstruction with microcolon?
A. Meconium Ileus
Meconium impacted at the terminal ileum. Linked to CF.
On WSCE - outlines meconium pellets obstructing terminal ileum.
B. Ileal atresia
On WSCE - Blind end of contrast column in ileum = Ileal atresia
C. Total colonic Hirschsprung disease
Main causes of neonatal distal intestinal obstruction with small distal /left colon?
A. Meconium plug syndrome (neonatal small left colon)
Nonpathological transient functional obstruction at the proximal descending colon**.
+/- if water souble contrast isnt therapeutic then - Rectal biopsy to exclude long segment HD
B. Colonic atresia
WSCE - blind ending small caliber distal colon (can be more proximal)
C. Hirschsprung’s disease
WSCE - Classically short rectosigmoid segment = small calibre rectum and dilated sigmoid (rectosigmoid ration <1).
Long segment - Transition zone from the small distal colon to the dilated proximal colon
**suction biopsy below this transition point.
Older child with distal intestinal obstruction or right iliac fossa pain/mass and normal appendix. Bilateral upper zone bronchiectasis ?
Distal intestinal obstruction syndrome
Secondary to CF
Older child meconium ileus equivalent
3 year old RUQ mass, hilar mass displacing the pancreatic head and causing intrahepatic duct dilatation. CT shows retroperitoneal lymphadenopathy?
Rhabdomyosarcoma
Most commonly arise form the CBD.
Expansile intraductal biliary mass in young child
Dilated intrahepatic bile ducts caused by downstream biliary obstruction
DDx - Choledochal cyst
Lobular liver mass in young patient with a central fibrous scar that doesn’t enhance?
Fibrolamellar carcinoma
Central scar (low T1 and T2 as fibrous) that doesn’t enhance or Ca2+ in scar help differentiate from FNH
Sacular dilatation of the proximal large intrahepatic biliary ducts, alongside focal strictures (beaded apperance) ?
Caroli Disease (Type V choledochal cyst)
AR disease linked with medullary sponge kidney, ARPKD and ADPKD and significant risk of cholangiocarcinoma
Port wine stain, recurrent seizures, Gyral cortical calcification and leptomeningeal enhancement ?
Sturge-Weber syndrome
Cerebral lobar atrophy with cortical/subcortical Ca⁺⁺ leptomeningeal enhancement
enlarged ipsilateral choroid plexus
What is the most common type of tracheoesophageal fistula ?
Type C
Type A - No/Absent TOF, just oesophageal atresia (9%)
Type B - oesophageal atresia with a fistula between the proximal pouch and the trachea
**Type C - oesophageal atresia with a fistula from the trachea or the main bronchus (Level of carina) to the distal oesophageal segment (80%)
**
Type D is oesophageal atresia with both proximal and distal fistulas
Type H - Isolated TOF with no oesophageal atresia.
Present later in life with recurrent URTIs
C and D: Gas in stomach & bowel
A and B: Gasless stomach & bowel
US measurements to Dx pyloric stenosis ?
Single wall thickness - 3mm
Pyloric transverse diameter: > 13 mm
Pyloric length - > 15mm
Pyloric volume > 1.4cm3
Overinflation of the apicoposterior segment of the left upper lobe with ‘gloved finger’ sign?
Bronchial atresia
Gloved finger - is mucal impaction (mucocele). Presents usually in adolescences
Ovoid/round or tubular branching mass (finger-in-glove sign) with hyperinflated lung distally
Transient tachypnoea of the new-born (TTN)
Nb - normal descend down the vaginal canal squeezes fluid out of the lungs.
Term baby
C-section
Diabetic mother.
CXR -
WET lungs - Looks like pulmonary oedema
Bilateral symmetrical nodular air space opacities or intersitial markings
Fluid in fissures
pleural effusions. No cardiomegaly
Peaks in severity in 24hrs, resolved by 3 days.
Surfactant deficient disease
(a.k.a. respiratory distress syndrome & hyaline membrane disease)
Pre-term neonate
Diffuse granular opacities
Lung volume appears low
Air bronchograms
DDx
Group B Streptococcal Pneumonia as ‘diffuse granular’ opacities + PLEURAL EFFUSIONS
Can go on to develop PIE and Bronchopulmonary dysplasia
-Varying degrees of hazy opacities, cysts, hyperinflation, & coarse linear opacities diffusely in premature infant
- 28 days needing 02 /respiratory suppport
Meconium aspiration syndrome
Term/post term
Coarse, rope-like perihilar opacities
hyperinflation in term infant
Air bronchograms ABSENT
Can develop PTx, pneumomediastinum or PIE
Congenital lobar overinflation
Check-valve bronchial obstruction.
Neonate with hyperexpanded lucent pulmonary lobe
Especially left upper lobe
May be fluid-filled in 1st few hours after birth
LUL commonest. Then RML
Bronchogenic cyst
clasically subcarinal
Malformation of bronchial tree and lined with respiratory epithelium so hence produce fluid.
**Do not usually communicate with bronchial tree so don’t contain air. **
If air = infected
Middle Mediastinal or Central location with NO feeding vessels
If small asymptomatic/incidental
Congenital pulmonary airway malformation
-multiple gas filled cystic mass in the lung,
-contralateral mediastinal shift.
Often seen antenatal scan but can present neonatal respiratory distress or milder cases in child with LRTI and decompensation.
Can be cystic and solid masses thus CTA is performed to rule out a feeding vessel (pulmonary sequestration)
**Not lobe predominant. **
Remember CLO upper lobe and sequestration left lower lobe
Pulmonary Interstitial emphysema
Alveolar rupture from barotrauma.
Prevalent in low birth weight, hyaline membrane disease, meconum aspiration after ventilation
New, “bubbly,” cystic or linear lucencies within lung of intubated premature infant
Can cause acute life threatening PTX and pneumomediastinum
Treated with high frequency oscillation ventilation
Sequestration
Both have arterial supply from systemic circulation/aorta
Extralobular less common (25%) vs intralobular (75%)
Extralobular has its own pleura and additional congenital abnormalities - Diaphragmantic hernia/VSD
Venous drainage is into the IVC - so can present with respiratory distress to shunting
Intralobular presents later in life with recurrent infection
No pleura lining. Drains into pulmonary veins
CXR
opacity adjacent to the heart, abutting the diaphragm.
5 Ts of neonatal cyanotic and plethoric congenital heart disease?
TGA
Tricuspid atresia
Truncus arteriosus
TAPVC
Tingle ie single right ventricle
Most common cyanotic congenital heart disease present after 12 months?
TOF
Nb - Boot shaped heart as the RVH turns the cardiac apex upwards.
Can have normal CXR when infant
Pulmonary oligemia
Strong association with right sided aortic arch
RVOT commonly implicated cause is infundibular stenosis at Pulmonary valve
Very large enhancing hemi thoracic mass in a 1-2 year old - 2 key differentials?
A. Pleuorpulmonary blastoma
-Large pulmonary cystic, solid, or mixed mass in child < 6 years old
B. Askin tumour/Ewing Sarcoma of Chest Wall
-Early teens with large unilateral intrathoracic mass
-Large unilateral lobular opacity; may occupy entire hemithorax, particularly in combination with pleural effusion
**–Key differentiation feature is rib destruction = Askin **
Child with incidental lucent lesion in the long bone, diameatphyseal, sclerotic margin, eccentric and elongated?
Fibrous cortical defect if <2 cm
Non-ossifying firboma if > 2cm
Common benign
15-year-old with lucent lesion in femur, permeative, periosteal reaction and associated soft tissue component?
Ewings sarcoma
Permeative/moth eaten appearance
Often Lamellated periosteal reaction can be sunburst
Predilection for DIAPHYSIS of long bones
1/3 have metastatic disease at presentation
Adolescent Painful leg, fever. Poorly defined aggressive lesion with sunburst periosteal reaction and fluffy cloud like osseous matrix?
Osteosarcoma
Predilections for proximal tibia or distal femur (around the knee) = METAPHYSIS
Staging need MRI of entire bone affected. Nuclear bone scan and a CT chest.
Parosteal - lower grade, older age (20-50s)
Telangiectatic - purely lytic, poor prognosis, fluid-fluid levels
Multicentric - Age 5-10mvery poor prognosis
Differentiate between multicystic dysplastic kidney and hydronephrosis ?
Hydronephrosis apparent cysts on US communicate with each other
Hydro is the most common cause of neonatal abdominal mass
Multicystic dysplastic kideny
-normal renal architecture is entirely replaced by a non-functioning mass of cysts separated from one another by fibrous tissue.
- ‘Bunch of grape’
Most common solid renal mass in neonate?
Mesoblastic nephroma
*Most common Soild neonatal renal mass**
US - large hypoechoic renal mass which cannot clearly be separated from the normal renal parenchyma. Get nephrectomy.
**Doesnt invade into the collecting system or veins **
Multicystic dysplastic kidney 2nd commnest SOLID .
Any renal mass commonest is hydronephrosis.
Key features of neuroblastoma?
Peak age is 2
Most adrenal in location, 1/3 retroperitoneum and 1/5 posterior mediastinum.
Secrete catecholamines = hypertension ir VIP = watery diarrhoea
**ENCASES the IVC **
Crosses midline
**Ca2+ **
‘Dropping lily sign’ on urography - kidney displaced Downard’s
**Staging is performed with an 123I-MIBG scan **
Stage IVs (disease involving the skin, bone marrow and liver) which has the best prognosis.
Key features of Wilms (nephroblastoma) tumour?
peak age 3-4
**Displaces +/- invades the IVC
**
less commonly crosses the midline or Ca2+
‘Claw sign’ - Rim of normal renal tissue extending around the mass.
10% tumour - Ca2+ ( curvillnear), vascular invasion, bilateral, lung mets
Abdominal mass in 4 year old boy, US mass of cysts with thick fibrous capsule. Mass protudes into the renal pelvis but no solid components?
Multilocular cystic nephroma
*Protusion into the renal pelvis
two age peaks; one at around age 4 which affects mostly boys and one at around age 40 which affects mostly women ie
‘Michael jackson’ tumour - like young boys and older women
Dense bones with short stature, acroosteolysis, increased angle of mandible
Pycnodysostosis
AR
widened hands and feet
Receding jaw and beaked faces -Obtuse mandibular angle
Osteosclerosis is dominant radiographic finding - Does not obliterate marrow space
DDx
Osteopetrosis - Whole medulla of long bone sclerotic
Shortening of long bones with widening of the metaphysis and diaphysis. Kyphosis and beaking of the anterior vertebral bodies.
Mucopolysaccharidosis
metacarpals that are short, broad, and constricted proximally (pointing proximally)
Marcocephaly
Dysplastic changes in the femoral head
Odontoid hypoplasia → atlantoaxial instability
**Murquio = anterior middle Vertebral beaking **
Hulers = anterior inferior vertebral body beaking
Periosteal reaction, coarse trabeculation, epiphysel plates are widened, fraying at the metaphysis side?
Rickets
paediatric liver tumours and a raised AFP
see chart
NAI fractures
Child less than 1 =
Skeletal survery, reported by 2 radiologists and repeated in 3 weeks
CTB
If positive need MRI brain and whole spine
interhemispheric SDH highest specificity for abuse of any intracranial injruy
Schizencephaly
Open-lip schizencephaly may result in large cystic cavity often mistaken for cyst
Closed-lip schizencephaly may be very small & subtle, mimicking transmantle GM heterotopia
transmantle GM heterotopia
-Column of grey matter extending from the subependymal to the pial surface.
DDx
porencephalic cyst
-intraparenchymal CSF-filled cavity that communicates with the ventricle and subarachnoid space.
-lined with ** gliotic white matter **
- previous insult - fetal life or early infancy , Brain destruction during perinatal period
Neuroglial cyst
- doesnt communicated with the lateral ventricle
- Nonenhancing, CSF-like parenchymal cyst with minimal/no surrounding signal abnormality
Features of moyamoya
Attenuated COW with multiple tiny basal ganglia (BG) flow voids on MR
Large vessel occlusion- supraclinoid ICA/COW; anterior > > posterior circulation
“Puff of smoke” (moyamoya in Japanese)
Cloud-like lenticulostriate and thalamostriate collaterals on angiography
grading of VUR
Grade I: Reflux into distal ureters
Grade II: Reflux into collecting system (without calyceal dilatation/blunting)
Grade III: All of the above, plus mild dilatation of the pelvis and calices
Grade IV: All of the above, plus moderate dilatation (clubbing of calices)
Grade V: All of the above, plus severe tortuosity of the ureter
Grades IV and V
require surgery to avoid renal scarring, renal impairment and/or hypertension.
