Pedia 👶 Flashcards
Gyral pattern is established at what week?
38 weeks
Pattern of Myelination: True or False
Back to Front
Caudad to Cephalad
Central to Peripheral
True
Sequence of DEVELOPMENT of the corpus callosum:
Genu>body>isthmus>Splenium
True
Sequence if MYELINATION of the Corpus Callosum:
Genu> body> isthmus
FALSE
Splenium> genu
(Posterior to Anterior)
Pattern of vascularity in Preterm neonatal brain wherein the penetrating arteries supply the periventricular regions by extending INWARD from the surface of the brain
VentriculoPETAL
Pattern of vascularity where in the blood flow goes AWAY from the ventricles extending into the brain from the lateral ventricles; the intervascular border is moved peripherally to parasagittal region, hence affecting the subcortical white matter and parasagittal region in hypoxic injury
VentriFUGAL
Type of asphyxia with INTACT autoregulation, hence, there is redistribution of blodd flow to the hypermetabolically deep gray matter structures resulting to INJURY predominantly in the WATERSHED zones of the cerebrum
Mild to Moderate asphyxia
Type of asphyxia characterized by loss of autoregulation resulting to injury of the vulnerable regions such as the deep gray matter and early or active myelinating fibers.
Profound Asphyxia
Effects of hypoxic ischemic injury in premature infants (< 36 weeks)
- Geminal matrix hemorrhage
- Intraventricular hge
- Periventricular White Matter Injury
Cranial US - preferred modality for evaluation
Germinal matrix hemorrhage extending into and distending the ventricles.
Grade III
Grade I - Mild; confined to the GM notch
Grade II - Intraventricular extension with no associated distention of the ventricle.
Grade IV- Hemorrhagic venous infarct in the periventricular white matter.
This hypoxic brain injury is due to the selective vulnerability of the periventricular white matter secondary to the ventriculoPETAL pattern of vascularity in the pretem infant.
Periventricular Leukomalacia
US: Echogenic lesion in the white matter adjacent to the atria of the lateral ventricles.
Destructive lesions PRIOR to 28-30 weeks results in cavitation (PORENCEPHALY) True or False?
True
*capacity to develop gliosis does not occur until 28-30 weeks.
PVL Grading characterized by INCREASE periventricular echogenicityin deep WM into SUBCORTICAL cysts.
Grade IV
Grade I : Increase periventricular echogenicity WITHOUT cystic formation persistent for > 7 days.
Grade II: SMALL periventricular cyst.
Grade III: EXTENSIVE periventricular cyst in occipital and frontoparietal regions.
Perinatal Arterial Ischemic Stroke occurs between 20 wks AOG to 28 postnatal days, commonly affecting the MCA and is usually seen in TERM neonates. True or False?
True
Most common cause of small amounts of Subarachnoid, subdural and intraventricular hemorrhages in the TERM New Born
Normal Delivery
MC congenital heart disease?
Bicuspid aortic valve
Edema which may be accompanied by hemorrhage within the SUBCUTANEOUS tissues typically seen after vaginal delivery. It is NOT LIMITED by sutures and resolves within a few days without complication.
Caput Succedaneum
SUBPERIOSTEAL HEMORRHAGE CONFINED BY SUTURES which may ba ssociated with skull fractures and epidural hematomas. Increases in size after birth and may calcify.
Cephalhematoma
Most common cause of neonatal brain abscess
- Citrobacter
- Serratia
- Proteus
Most common form of pediatric CNS infection
Bacterial Meningitis
Neonate: GBS, E. coli
>1 yo: HiB, S. pneumonia, E. coli and N. meningitides
Typical imaging findings of CNS VIRAL INFECTION
US: Echogenic
CT: HYPOdense
MR: T2/FLAIR HYPERintense
Earliest sign og VIRAL encephalitis on MRI
Restricted diffusion in DWI
Often caused by REACTIVATION of previous OROFACIAL INFECTION with the following imaging findings:
MR: unilateral/bilateral T2/FLAIR HYPERintense signals in the MIDDLE TEMPORAL lobe
CT: HYPOdensities in the TEMPORAL lobe and INSULAR CORTEX
Herpes Simplex Virus 1 encephalitis
-leptomeningeal and cortical enhancement and focal calcification and hemorrhage may also be present
ATROPHY and resultant ventricular and SAS prominence associated with SUBCORTICAL and BASAL GANGLIA CALCIFICATIONS
HIV encephalitis
Focal or multifocal PERIVASCULAR INFLAMMATORY and DEMYELINATING disorder presenting as T2 HYPERintense signals with corresponding T1 HYPO- to ISOintense signal abnormalities with variable pattern of enhancement (solid, ring-like, or none), affecting both gray and white matter. History of RECENT VIRAL illness or VACCINATION is key in diagnosis
Acute Demyelinating EncephaloMyelitis (ADEM)
Caused by REACTIVATION of MEASLES virus years after infection presenting as T2/FLAIR HYPERintense signals in the cortex and basal ganglia
Subacute Sclerosing Panencephalitis
Cause of intractable epilepsy in children, presenting @ 1-15 yo. Imaging shows
1. HEMISPHERIC atrophy
- T2/FLAIR hyperintense signals in the FRONTAL and TEMPORAL lobes (predominantly WM regions) and in the BASAL GANGLIA
RASMUSSEN’S encephalitis
Bacterial and Viral infections are most commonly transmitted intrapartum and post-natally. True or False
False. Bacterial and FUNGAL
Infections most commonly transmitted in utero
- Syphilis
- Rubella
- CMV
- Toxoplasma
Infections acquired during the 1st trimester cause severe congenital malformations, while that acquired during the 3rd trimester cause destructive lesions. Tue or False
True
Most common serious viral infection in the New Born associated with polyMICROGYRA, especially in the Sylvian Fissure
Cytomegalovirus (CMV)
Congenital viral infection characterized by the following imaging findings:
- Periventricular/subependymal calci or hge.
- Ventricular or Sulcal prominence
- Periventricular and subcortical white matter changes
Cytomegalovirus (CMV)
Splenium should be as thick as the genu at what age?
1 yo
The lateral ventricles have parallel orientation, with posterior dilation (Colpocephaly), upturned anterior horns, and interhemispheric sulci extending up to the 3rd ventriclular margin with associated underrotation of the hippocampi
Complete Agenesis of Corpus Callosum
Seen as truncation of the anterior-posterior dimension of the corpus callosum
Partial Agenesis/ hypogenesis of the Corpus Callosum
Texas Longhorn appearance of the lateral ventricle is seen in this condition
Complete Agenesis of the Corpus Callosum
Partial agenesis of the Corpus Callosum is frequently associated with these interhemispheric lesions
Interhemispheric Cyst or Lipoma
Optic Nerve dysplasia is most reliably diagnosed on
Ophthalmologic Examination
Malformation caused by abnormalities in differentiation and midline cleavage of the prosencephalon on 5th week AOG. Hallmark: abnormal communication of the gray and/or white matter across the midline. There is facial dysmorphism (80%) (hypotelorism, cyclopia, cleft lip/palate)
HOLOPROSENCEPHALY
Gene mutation involving ciliary proteins characterized by vermian hypoplasia, thickening and elongation of the superior cerebellar peduncle (MOLAR sign) clinically manifesting as “panting” or “laughing” respiration with occulomotor apraxia in infancy
Joubert Syndrome
Characteristic enlargement of the ventricular atria
Colpocephaly
Complete absence of cleavage with anterior displacement of the cerebral tissue into “PANCAKE” configuration, large MONOVENTRICLE expanding posteriorly into a dorsal cyst.
Alobar Holoprosencephaly
Lack of separation of the posterior frontal and parietal regions with separation of the anterior frontal and the occipital lobes
SYNtelencephaly
Triad of enlarged posterior fossa, cystic dilation of 4th ventricle and vermian hypoplasia/agenesis.
Dandy-Walker Malformation
Majority of the Neuronal MIGRATION MALFORMATION occurs at what gestational week?
Between 12-24 weeks
A severe in utero brain injury which is attributed to bilateral internal carotid artery occlusion that often result to fetal demise?
Hydranencephaly
Absence of all brain parenchyma supplied by carotid arteries, with preservation of the posterior fossa structures and medial temporal lobes.
Treatment: CSF DIVERSION WITH VENTRICULAR SHUNT
Hydranencephaly
Distinct pattern of WHITE MATTER INJURY in utero (bet 24-34 weeks GA) caused by damage of OLIGODENDROCYTE PRECURSOR CELLS due to HYPOXIA
Periventricular Leukomalacia
This PATTERN WHITE MATTER INJURY shows a symmetric loss of periventricular white matter surrounding the TRIGONES of the lateral ventricles and shows a characteristic ANGULAR morphology of adjacent lateral VENTRICLES
Periventricular Leukomalacia
Also known as WHITE MATTER INJURY OF PREMATURITY
US: hyperechoic in the WM bordering the bodies of the lateral ventricles
MR: Punctate foci of abnormal hyperintensity on T1WI
Periventricular Leukomalacia
Characterized by Hemicerebral ATROPHY, IPSILATERAL compensatory osseous HYPERtrophy, and Enlargement of the FRONTAL SINUS
Dyke-Davidoff-Masson Syndrome
Characterized by Hemicerebral ENLARGEMENT, Ipsilateral osseous hypertrophy and paradoxical ENLARGEMENT of the LATERAL VENTRICLE, and thickened and ill-defined cortical ribbon?
A. Rassmussen Encephalitis
B. Dyke-Davidoff-Masson Sundrome
C. Hemimegalencephaly
D. HIV encephalitis
HEMIMEGALENCEHALY
Characterized by HEMICEREBRAL ATROPHY, T2/FLAIR HYPERintense signals in he FRONTAL and TEMPORAL lobes, as well as in the BASAL GANGLIA?
A. Rassmussen Encephalitis
B. Dyke-Davidoff-Masson Sundrome
C. Hemimegalencephaly
D. HIV encephalitis
RASMUSSEN Encephalitis
complete failure of division of the promesencephalic vesicle. A monoventricle is identified with “kissing choroid” by ultrasound. The thalami and basal ganglia are fused. The falx, corpus callosum, and interhemispheric fissure are absent
Alobar Holoprosencephaly
Fetal vermis is not completely developed until how many weeks gestation?
18 weeks AOG
It is characterized by fluid collection posterior inferior to the vermis and COMMUNICATES with the 4th ventricle. The vermis is typically intact. The tentorium is ELEVATED. No connection with the subarachnoid space is noted.
a. Dandy-walker
b. mega cisterna magna
c. Chiari Malformation
d. Arachnoid Cyst
Blake’s Pouch Cyst
Retrocerebellar cyst that COMMUNICATES with the 4th ventricle. The POSTERIOR FOSSA is ENLARGED, with an elevated tentorium. The vermis is INCOMPLETE, elevated, and rotated (>180 deg):
a. Dandy-walker
b. mega cisterna magna
c. Chiari Malformation
d. Arachnoid Cyst
Dandy-Walker Malformation
Cystic lesion taht amy develop in the posterior fossa, causing mass effect in the otherwise NORMAL VERMIS. It does NOT COMMUNICATE with the 4th ventricle and the tentorium MAY BE elevated.
a. Dandy-walker
b. mega cisterna magna
c. Chiari Malformation
d. Arachnoid Cyst
Arachnoid Cyst
*MC locaion: MIDDLE cranial fossa (50-60%)
other common location:
Retrocerebellar
Galassi classification of middle cranial fossa arachnoid cysts characterized by small, spindle-shaped, limited to the anterior portion of the middle cranial fossa, below the sphenoid ridge, and FREELY COMMUNICATING with the subarachnoid space. This type of represents the most common type of arachnoid cyst.
A. Type I
B. Type II
C. Type III
D. Type IV
Type I
Type II: extends to the Sylvian Cistern, displaces the temporal lobe with slow communication to subarachnoid space
Type III: LARGE, fills the whole middle cranial fossa, w/ midline shift, little communication with subarachnoid space
Results from arrest of migration of neuroblasts with abnormal cortical lamination and failure of sulcation
Agyra/ Pachygyra
This results from excessive folding of the cerebral cortical cells layers w/ fusiosn of the gyral surface. It is common after CMV infection in the 2nd trimester. It appears as mulitple SMALL, IRREGLAR SULCI.
Polymicrogyra
This is the leading infectious cause of sensorineural hearing loss. It is cahracterized by periventricular and subependymal calcifications. Ct may demonstrate Mondini malformation.
CMV infection
*Mondini Malformation
- absent interscalar septum
- large vestibule
- enlarged vestibular aqueduct
Maternal infection with toxoplasmosis after 20 weeks AOG has LESSER rate of transmission to fetus with less severe sequela such as blindness, epilepsy, and mental retardation.
FALSE; much HIGHER rate of transmission
The following are imaging findings of congenital TOXOLASMOSIS except:
a. Non-shadowing cerebral and hepatic calcifications
b. intracranial calcifications mau be periventricular or random distribution.
c. Subependymal cysts
d. “Candle stick” sign
e. None of the above.
None of the Above
Findings of pituitary hypoplasia, absent septum pellucidum, and optic dysplasia:
A. Syntelencephaly
B. Septo-optic Dysplasia
C. Semilobar Holoprosencephaly
D. Lobar Holoprosencephaly
Septo-Optic Dysplasia
P-A-O
P ituitary hypoplasia
A bsent septum pellucidum
O ptic dysplasia
Refers to the dilation of the ventricular trigone and occipital lobes due to agenesis of the corpus callosum
Colpocephaly
Texas longhorn, Viking Helmet, Race Car appearance
Corpus Callosal Agenesis
Most severe form of malformation due to generalized ABNORMAL transmantle MIGRATION. The brain is smooth with hour glass shape due to mild infolding of the Sylvian fissure
Lissencephaly
It results from the DISRUPTION of the normal TERMINAL neuronal MIGRATION resulting in an increased in small gyri; associated thickened dysplatic overlaying of leptomeninges
Polymicrogyra (PMG)
- often BILATERAL
Most common location of Polymicrogyra
PeriSylvian Cortex
Abnormal Gray matter-lined cleft extending from the ventricular ependymal surface to the pial cortical surface. The cleft is lined by polymicrogyra, extending full length of the cleft.
SCHIZENCEPHALY
Open Lip - communicates w/ ventricle
Close Lip - cleft walls closely appose
Unilateral schizencephaly is often associated with contralateral periSylvian polymicrogyra (PMG). True or False
TRUE
*Porencephaly vs Schizencephaly
Porencephaly = lined by white matter
Schizencephaly = lined by PMG
Trapped nest of gray matter within the brain, which appear as variable sized nodules that are isointense to gray matter in all sequences.
Heterotopias
Does NOT enhance or CALCIFY
Results from early ARREST of MIGRATION. Double Cortex syndrome. Symmetric band of gray matter is separated from the overlying cortex by a thin band of white matter producing a three-layer cake appearance.
Band Heterotopia
*Lissen = smooth agyric, thick cortex
*Band heterotopia= thin cortex, multiple gyri, shallow sulci
Results from abnormal PROLIFERATION and DIFFERENTIATION of neurological precursors:
a. Band heterotopia
b. Focal cortical dysplasia
c. Schizencephaly
d. Lissencephaly/Pachygyra
Focal Cortical Dysplasia (FCD)
*Histo Hallmark:
Lack of normal cortical lamination
* Subcortical HYPERINTENSE T2 w/ adjacent subtly thickened cortex
FCD types
Type I = Alteration in cortical layering
Type II = Cortical dyslamination/ Dysmorphic neurons
Type III = Encephaloclastic lesion
True
*FCD present as intractable epilepsy
Radial extension of the balloon cells and ectopic neurons into the deep white matter seen in type I FCD
Transmantle Sign
Differential diagnosis for posterior fossa cyst
D andy walker Complex
A arachnoid cyst
M ega Cisterna Magna
B lake’s pouch cyst
Angle formed by 2 lines along the anterior surface of the vermis and the dorsal surface of the brainstem; Normal is <18 deg
Tegmento-Vermian Angle
Line drawn horizontally from the fastigium (dorsal point of the 4th ventricle) and most dorsal point of the vermis taken in sagittal view
Fastigium-Declive line
posterior fossa = enlarged
vermis = hypoplastic
T-V angle = >18
Classic Dandy-Walker Malformation
Posterior fossa = Normal
Vermis = hypoplastic
T-V angle = >18
Vermian Hypoplasia
Posterior fossa = Normal
Vermis = Normal
T-V angle = >18 deg
Blake Pouch Remnant
*Due to non-perforation o fthe foramen of Magendie
Posterior fossa = Normal
Vermis = Normal
T-V angle = Normal
Mega Cisterna Magna
*Normal size Cisterna Magna
2-10 mm
Largest of the subarachnoid cisterns
Cisterna Magna
a.k.a cerebellomedullary cistern
Midline fusion o fthe cerebellar hemispheres & absence of vermis; best seen in coronal view as cerebellar folia that is contiguous across the midline
Rhombencephalosynapsis
Failure of decussation of the superior cerebellar peduncles; superior cerebellar peduncles appear thickened Molar Tooth appearance
Joubert Syndrome
Increased/prominent inner table convolutions, sellar erosions, and enlargement.
Copper Beaten Skull
- seen in:
Craniosynostosis
Hydrocephalus
Hypophosphatamia
Closing of fontanelles:
Posterior Fontanelle
Anterior Fontanelle
Metopic Suture
2- 3 months - post fontanelle
18 months - ant fontanelle
1 yo - metopic suture
Closed suture: Sagittal
Head shape: Long, narrow
Scaphocephaly/ Dolicocephaly
Closed suture: Bilateral Coronal &/or Lambdoid
Head shape: Short, wide,
Associated Syndromes:
Apert
Carpenter’s
Cornelia
Down’s
Brachycephaly/ Bradycephaly
Closed suture: Metopic
Head shape: Frontal wedging, KEEL
TrigOnocephaly
- Omega sign
TriM (TrigonoMetopic)
Closed suture: All
Head shape: Tower-like skull
Associated with:
* 8thcranial nerve lesion
* optic nerve compression
* mental deficiency
* syndactyly
Turricephaly
- most severe form
Closed suture: Sagittaal, coronal, Lambdoid
Head Shape: Cloverleaf skull/
Cloverleaf skull/ Kleeblattschadel
Most common primary craniosynostosis
Scaphocephaly/ Dolicocephaly
*bitemporal pinching
2nd most common craniosynostosis
Brachycephaly
*premature closure of bilateral coronal and lambdoid sutures
Sphenoid is drwan upward toward the closed suture; seen in PLAGIOCEPHALY
Harlequin’s Eye
Most common anomaly associated with craniosynostosis:
Limb Defects
*syndactyly
*polydactyly
Bicoronal synostosis associated with symmetric syndactyly of the 2nd to 4th digits resulting to Mitten or Paddle hand
Apert Syndrome
(Apir 🙏; Apert, paddle hand)
Brachycephaly, facial dystosis with hooked PARROT NOSE and small maxilla, bilateral exopthalmos, and genetic transmission
Crouzon Syndrome
Not associated with increased ICP; appear as soap bubble rarefractions of the upper calvarium; fade after birth; Disappears after 4-5 months of age
Lacunar skull
a.k.a Lückenschädel skull
Soft, fluctuant, red-to-blue scalp mass over the sagittal or transverse sinuses;
Cranial venous anomalyin which there is an abnormal communication between intracranial dural sinuses and extracranial venous structures, usually via an emissary transosseous vein
Sinus Pericranii
Most common phakomatosis
Neurofibromatosis Type 1
- a.k.a Peripheral Neurofibromatosis
C A F E S P O T
C afe au lait spots
A axillary or inguinal ferckling
F ibromas (2 or more) or plexiform (1)
S keletal abnormalities (leg bowing)
P ositive family hx
OT optic tumor (optic gliomas) Lisch nodules
Characteristic/pathognomonic lesion of NF1; presenting as “bag of worms”
Plexiform Neurofibromas
Central neurofibromatosis with characteristic “bilateral acoustic Schwannoma” and dorsal nerve roots in dumbbell configuration.
Neurofibromatosis Type 2
*M I S M E
M ultiple
I nherited
S chwannomas
M eningiomas &
E pendymomas
Characterized by hamartomas within multiple organ system with epilepsy as the MC neurologic symptom. dermal manifestation includes ADENOMA SEBACEUM.
Tuberous Sclerosis
*Vogt Triad
1. Seizure
2. Adenoma sebaceum
3. Mental retardation
MC lesion seen in TS
Subependymal Hamartomas
*DDx:
Gray matter heterotopia (does not calcify)
Autosomal dominant. Consist of retinal ANGIOMAS, and cerebellar and spinal HEMANGIOBLASTOMA
Von Hippel Lindau Syndrome
- associated with
Renal/ Heaptic Cysts
RCC, Pheochromocytoma
Hemagioblastoma (cyst w/ murallll nodule in the cerebellum)
Encephalotrigeminal angiomatosis; angiomatous lesions of the skin and meninges; Facial port-wine stain; Pathology in the brain: PIAL ANGIOMATOSIS and ipsilateral CHOROID PLEXUS Hypertrophy
Sturge-Weber Syndrome
Cutaneous lesion of NF1 except:
a. cutaneous neurofibromas
b. portwine stain
c. Lisch nodules
d. cafe au lait spot
Port-wine stain - Sturge Wr Syndrome
Cutaneous lesion of Sturge-Weber:
a. cutaneous neurofibromas
b. portwine stain
c. Lisch nodules
d. cafe au lait spot
Port-Wine stain (facial)
Cutaneous lesion of tuberous sclerosis:
a. cutaneous neurofibromas
b. adenoma sebaceum
c. Lisch nodules
d. cafe au lait spot
Adenoma sebaceum
True of supratentorial embryonal tumor except:
a. well-defined lesion & may be solid homogeneous or heterogenous w/ cyst formation
b. calcifies
c. w/ necrosis and hge
d. leptomeningeal dissemination and drop mets are frequent
e. none of the above
None of the above
Lesion that is ALMOST ALWAYS INTRAventricular; commonly seen in adults; DOES NOT usually ENHANCE; WITHOUT perilesional edema
Subependymoma
MC location of Subependymoma
Frontal horn of lateral ventricle (near foramen of Monro
followed by:
fourth ventricle
septum pellucidum
MC seen in children under 3 YO; intraventricular in location w/ transependymal extension; WITH perilesional edema; EXTRUDES into the foramens of LUSCHKA and MAGENDIE.
EPENDYMOMA
MC location of ependymoma
Posterior Fossa (4th ventricle)
MC choroid plexus tumor
Choroid plexus Papilloma
MC location children: trigone lat vent
MC location adult: 4th ventricle
Slow-growing, benign, non-glial tumors that arise from the etoderal remnant of the Rathke’s Pouch in the sellar/suprasellar region. WHO Grade I
Craniopharyngioma
*Chidren: Adamantinomatous; encases vessels;calcifies
*Adult: Squamous papillary variant
MC posterior fossa tumor in childhod
Medulloblastoma
*Classic - MC; cental post fossa (fills the 4th ventricle)
*Large cell-anapastic - most maliganant
Vascular structure engulfed by Diffusse Intrinsic Pontine Glioma
Basilar artery
MC tumor in children; CYST W/ enhancing MURAL NODULE
Pilocytic Astrocytoma
*COMMONLY arises in the CEREBELLUM
MC pineal gland tumor
Pineal Germinoma
Pineal tumor that is hyperdense on CT with homogeneous enhancement; central calcification ENGULFED PATTERN
Pineal Germinoma
*compresses the tectal plate resulting in PARINAUD SYNDROME; “upward gaze” palsy
Malignant, unencapsulated tumor of the pineal gland, CALCIFIES less than pineocytoma that is PERIPHERAL in location EXPLODED PATTERN w/ lateral displacement of the pineal gland
Pineoblastoma
MC benign lesion in the pineal region
Pineal Cyst
MC cause of lobar pneumonia
S. pneumoniae
MCC of NEONATAL pneumonia
Group B hemolytic Streptoccoccus Pneumonia (Lobar pneumonia)
Multiple bilateral patchy alveolar opacities is suggestive of
Bacterial infection
MCC of interstitial pattern (hazy, reticular, or reticulonodular) in the lung of a child
Viral or Mycoplasma infection
The following pulmonary conditions predominate in the lower lungs except:
a. Tuberous Sclerosis
b. Langerhans cell histiocytosis
c. Connective tissue disease
d. Primary interstitial pneumonia
Langerhans cell histiocytosis
Pulmonary underdevelopment presenting as ABSENT bronchus, pulmonary parenchyma, and pulmonary arteries
Pulmonary AGENESIS
Pulmonary underdevelopment presenting as RUDIMENTARY bronchus with ABSENT pulmonary parenchyma and pulmonary arteries.
Pulmonary APLASIA
Pulmonary underdevelopment presenting with hypoplastic bronchus and pulmonary arteries with VARIABLE amounts of pulmonary parenchyma
Pulmonary HYPOPLASIA
Result from proximal arrest of the bronchial branching; Characterized by peripheral bronchioles (near the pleura) containing cartilage in their walls; hypoplasia or absence of the ipsilateral pulmonary artery
Congenital Pulmonary Hypoplasia
Hypoplastic lung DRAINED by an ANOMALOUS RIGHT PULMONARY VEIN seen as a curvilinear vertical vein extending along the right heart border emptying into the IVC.
Hypogenetic lung or SCIMITAR Syndrome
*right lung is the hypoplastic lung
Anomalous PV usually drains into the IVC
Curvilinear vertical vein extending along the right heart border emptying USUALLY into the IVC. Classic vertically oriented curvilinear density projecting over the right hemithorax
Scimitar Sign
*Scimitar vein may also drain in:
hepatic vein
portal vein
azygous vein
coronary sinus
right atrium
MCC of pulmonary hypoplasia due to INTRATHORACIC compression
Congenital Diaphragmatic hernia
*Pumonary insufficiency
-Most significant cause of morbidity and mortality
MCC of pulmonary hypoplasia secondary to EXTRATHORACIC compression
OLIGOHYDRAMNIOS WITH RENAL DISEASE
*Potter syndrome -failure of kidney to develop
*Bilateral cystic kidney
*Obstructive uropathy
ACQUIRED pulmonary hypoplasia secondary to OBLITERATIVE bronchiolitis resulting to AIR TRAPPING; Unilateral HYPERlucent lung
Swyer James Syndrome
*lung size DOES NOT change with respiration
*congenital pulmonary hypoplasia changes lung size during respiration
Consist of a large posterior right-sided aortic arch and small anterior left-sided aortic arch encasing the trachea and esophagus
Double aortic arch
*Lateral Radiograph
1. increased retrotracheal density
2. Anterior bowing of trachea
3. Tracheal narrowing
Components of vascular ring
- Right ascending aorta
- Aberrant left subclavian artery
- Ligamentum arteriosum or persistent Ductus Arteriosus stretching from the LSA to the PA anterior to the trachea
LEFT PA ARISES from the RIGHT PA and courses lateraly to the left side in b/w the trachea and esophagus compressing the trachea posteriorly
Pulmonary SLING anomaly
*Types:
Type 1- normal located carina
Type 2- inferior located carina; MC
Thin-walled lung cavities that commonly occur with pulmonary infection in children
Pneumatocoele
Results from early maldevelopment of the airway; there is abnormal bronchial communication or bronchial atresia; stomach is normal in location.
Congenital Pulmonary Airway Malformation (CPAM)
a.k.a Congenital cystic adenomatoid malformation
*Type 1 = Uniform air cyst, >2cm; MC
Type 2 = Variable size <2cm
Type 3 = MICROcyst
MC location of congenital diaphragmatic hernia
Foramen of Bochdalek
*posterolateral, left side
*solid viscera MC herniates through the right side
The ff are true of surfactant deficiency dse except:
a. MC of death in preterm infants
b. occurs in fullterm infants of DM mothers
c. both structural and functional immaturity
d. None of the above
None of the above
Complication of positive airway pressure treatment usually for SDD. Results from increased capillary permeability due to oxygen toxicity resulting to leakage of lfuid into the pulmonary interstitium.
Leaky Lung Syndrome
Abnormal development of the pulmonary lymphatic system resulting in dilation and obstruction of the lyphatic channels; patiet present with REFRACTORY PLEURAL EFFUSION
Pulmonary Lymphangiectasia
Bilateral pleural effusion is most commonly seen in
Renal disease
(e.g. AGN, nephrotic syndrome)
MCC of massive pleural effusion in neonates; usually UNILATERAL
Chylothorax
*caused by traumatic tear or congenital defect of the thoracic duct
MCC of PULMONARY MASS in CHILDREN
PSEUDOTUMOR
- from round pneumonia
others:
pulmonary abscess
MC TRUE lung mass in children
Post-inflammatory granuloma due to TB or fungal infection
Reactive lesion developing from healing pneumonia; solitary; well-defined; peripheral; predilection to the lower lobes
Inflammatory myofibroblastic tumor
MC foregut duplication cyst
Bronchogenic Cyst
Subcarinal = MC location
*does NOT communicate w/ airway
*Cyst in the RIGHT paratracheal or subcarinal region
Mass of lung tissue W/O connection to the bronchial tree and is supplied by SYSTEMIC ARTERY
Pulmonary Sequestration
*MC in the left side
*INTRAlobar - MC type; child/teens
*EXTRAlobar - newborns; w/ own pleura; unaerated; drains via azygos/hemiazygos
Triad of bronchial atresia
- Bronchocoele
- Hyperlucent distal lung
- Hypoperfusion of the distal lung segment
MC MALIGNANT neoplasm of childhood
Metastasis
MC childhood tumors to metastasize to the lungs
W ilm’s tumor
O steosarcoma
R habdomyosarcoma
E wing Sarcoma
*Neuroblastoma metastasize to the LIVER
MCC of apparent anterior mediastinal mass
Thymus
Faulty development of the 3rd and 4th pharyngeal pouches resulting to:
Thymic aplasia, absent parathyroid, CV abnormalities
DiGeorge syndrome
Anterior mediastinal masses except:
a. teratoma
b. neuroenteric cyst
c. dermoid
d. cystic hygroma
neuroenteric cyst
MC MIDDLE mediastinal mass
Lymphadenopathy
*massive adenopathy = lymphoma/leukemia
*unilateral adenopathy = TB
Primary subpleural lesion in the mid to lower lobes commonly seen in PTB
Ghon Focus
Calcified subpleural lesion (Ghon focus) with associated ipsilateral lympadenopathy
Ghon Complex
Calcified Ghon Focus and hilar lymph nodes
Ranke Complex
MC origin of POSTERIOR mediastinal mass
Neurogenic
*Clues:
Pedicle erosion
Interpedicular/ rib space widening
Bone erosion
Terrible ‘T’s of anterior mediastinum
Thymoma
Terrible lymphoma
Thyroid tumor
Teratoma
MC malignancies to involve the chest wall in children
- Ewing sarcoma
- PNET (Primitive NeuroEctodermal Tumor; Askin tumor)
*Large extrapleural soft tissue mass w/ rib destruction and pleural effusion
Metastatic RIB lesion common in infants and children are lesions from
Neuroblastoma
Useful in assessing location, size, and flow pattern of aortic and ductal arches; Can evaluate arch sidedness
Three Vessel Tracheal View
Indication most predictive of cardiac disease
Abnormal Four-chamber view on routine obstetric ultrasound
Asymmetric Pulmonary Vascularity is seen in
- TOF = Diminished flow to the left lung
- TA = Diminished flow to 1 or both lungs
- Valvular PS = Preferential FLOW to left
Causes of enlarged PA
- Increased pulmonary blood flow
- Post stenotic dilation
- Pulmonary valve insufficiency (regurgitation)
Causes of small or absent PA
- Pulmonary outflow tract obstruction
- Abnormal position of PA (PTA, TOGA)
MC abnormality in the contour of the heart
Coarctation Of the Aorta
figure of 3 sign
Most often an isolated anomaly; if accompanied by CHD, seen with PTA and TOF. * “Reverse S” * configuration in barium esophagogram.
Right-sided Aortic Arch
*may be a clue in the presence of a vascular ring
ACYANOTIC heart disease with INCREASED pulmonary blood flow
VSD
ASD
Aortic Pulmonary Window
PDA
MC congenital heart abnormality AFTER bicuspid aortic valve
VSD
MC type of VSD
Perimembranous
*near junction o fthe membranous and muscular portions
MC type of VSD associated with TOF, PTA and AVSD
High in membranous septum
Characteristic findings of:
Elarged PA, Left-sided cardiomegaly, and Increased pulmonary vascularity
VSD
MC type of ASD
Ostium SECUNDUM
*defect @ the center of fossa ovalis
Results form abnormal development of the endocardial cushion forming the interatrial and interventricular septum. Usually large defect seen anterior to the fossa ovalis @ the base of the atrial septum.
Ostium Primum ASD
*commonly seen in Trisomy 21
Increased pulmonary vascular resistance from conversion of a long-standing acyanotic left-to-right shunt into cyanotic right-to-left shunt.
Eisenmenger Syndrome
There is Right-sided cardiomegaly with RV volume overload, small aorta, diastolic bowing of the septum to the left, with NON-enlarged LA
ASD
*non enlarged LA, distinguishing feature of ASD from other left-to-right shunt
Results from failure of complete division of the prmitive truncus arteriorsus which leaves a c ommunication b/w the aorta and PA JUST ABOVE the valves
Aortopulmonary Window
Characterized by:
* Dilated PA
* Enlarged LA, LV (Left-sided cardiomegaly)
* Increased pulmonary vasculature
* Enlarged proximal aorta
Patent Ductus Arteriosus
*normally closes after birth
*persist as complication of hypoxia in premature infants
CYANOTIC heart dse with INCREASED pulmonary vascularity
TOGV ( D and L transposition)
TAPVR
PTA
DORV
SV
(kulang sya sa oxygen kaya TTPiDS sya)
MC form of CYANOTIC heart disease with INCREASED pulmonary blood flow
Complete Transpostion Of Great Vessel (TOGV D- transposition)
*position of aorta and PA are reversed
*needs an ASD, VSD, or PDA to survive
MC CYANOTIC heart disease
Tetralogy of Fallot (TOF)
Cardiomegaly with an ovoid appearance; EGG on a STRING appearance; anteriorly placed aorta results in increased RETROSTERNAL opacity.
Transposition Of Great Vessels - Complete (D transposition)
Dx is suggested by prominence along the left upper cardiac border representing the right ventricular outflow tract with a left-sided aorta.
Transposition of the Great Vessels - Corrected (L transposition)
Cyanotic heart disease with increased pulmonary blood flow wherein the AORTA is anterior and lateral to PA and ARISES from the RV.
Double Outlet Right Ventricle (DORV)
Type I = Subaortic VSD; MC; covers Ao
Type II = subpulmonic VSD; covers PA
TYpe III = doubly committed; covers both Ao and PA
Type IV = not committed; inferior to the IV septum
MC type of TAPVR
SUPRACARDIAC - Type I
*empties into a large SUPRACARDIAC VEIN (perisistent left SVC, left brachiocephalic vein, Right SVC, azygos vein)
Venous drainage of Type II TAPVR (Cardiac)
Right Atrium
Coronary sinus
Venous drainage of Type III TAPVR - Infracardiac
Portal Vein
*common vein travels into the esophageal hiatus and drains into PV, or less commonly an abdominal vein
Classic SNOWMAN appearance on radiograph due to prominent superior mediastinum from large inverted U-shaped vessel emptying to the SVC
Total Anomalous Pulmonary Venous Return (TAPVR)
*types 1 & 2 overload the right side of the heart
Failure of the truncus arteriosus to divide into PA and Aorta with both vessel being fed by a singel vessel that overrides a high VSD; seen as CONCAVITY in the usual site of PA
Persistent Truncus Arteriosus
Type I = MC; Ao and PA arise from same trunk
MC PTA where both Aorta and PA arise FROM a COMMON trunk.
Type I PTA
PA arises separately from the posterior aspect of the trunk just above the truncal valve
Type II PTA
*type III - least common; arise independently from either side of the trunk
Type IV - Neither arise from the common trunk
Group of anomalies in w/c one ventricle is rudimentary and the other is large and the only functional
Single Ventricle
*MC underdeveloped RV
MC chd to cause DIMINISHED pulmonary vascularity; MC cause of CYANOTIC heart disease
Tetralogy of Fallot
*TOGV - MC cyanotic heart disease WITH increased pulmonary vascularity.
Classic finding of BOOT-shaped heart sec to RVH; decreased pulmonary vascularity; combination of right-sided aorta and decreased pulmonary vascularity is highly suggestive
Tetralogy of Fallot
Egg on a string
a. DORV
b. TGV
c. TAPVR
d. TOF
TGV
Snowman Configuration
a. DORV
b. TGV
c. TAPVR
d. TOF
TAPVR
BOOT-shaped heart
a. DORV
b. TGV
c. TAPVR
d. TOF
TOF
Cyanotic CHD with decreased pulmonary vascularity consisting of:
*Tricuspid valve atresia
*Pulmonary atresia/stenosis
*Underdeveloped RV
The PA shadow appears flat with enlarged RA
Hypoplastic RIGHT heart syndrome
Cyanotic chd with decresaed pulmonary vascularity consisting of:
*TRicuspid valve atresia
*Pulmonary valve atresia/stenosis
*Hypoplastic RV
The PA is flat with enlarged RA
Hypoplastic RIght Heart Syndrome
Type of PA w/ ABSENT PA and is supplied by major AORTOpulmonary collaterals
Type C
*Type A- supplied by pdA
*Type B - supplied by Both native PA and major Aortopulmonary window collaterals
Consist of malformed tricuspid valve that is displaced downward, resulting in ATRIALIZATION of the RV.
Ebstein Anomaly
*classic “BOX-shaped” heart
Classic “BOX-shaped* heart
a. DORV
b. Ebstein Anomaly
c. TAPVR
d. TOF
Ebstein Anomaly
Focal and complex ABSENCE of the RV myocardium; the RV wall appears THIN
UHL anomaly
MC affected valves in congenital cardiac valves STENOSIS
SEMILUNAR VALVES
*Aortic
*Pulmonic
Cardiac valve stenosis associated with POST-STENOTIC dilation of PA with prominence of LEFT PA and INCREASED pulmonary blood FLOW to the LEFT lung.
VALVULAR Pulmonic Stenosis
*ONLY VALVULAR forms of aortic and pulmonic stenosis are associated with post-stenotic dilation
MC type of aortic stenosis
SUBvalvular Aortic Stenosis
Tyoe of aortic stenosis associated with WILLIAMS Syndrome
SUPRAvalvular Aortic Stenosis
MC form of SUBvalvular Pulmonic stenosis associated with TOF
INFUNDIBULAR
Form of COA presenting w/ PRE and POST STENOTIC dilations resulting in figure of 3 sign; Stenosis is seen @ or just distal to the level of DA
Juxtaductal COA
*collaterals:
1. intercostal arteries (T4-T8) rib notching
2. Internal mammary arteries
3. Arteries around the scapula
Aortic arch appears tortuous, dilated and kinked, WITHOUT evidnece of pressure gradient across the kinking
Pseudocoarctation of the Aorta
Condition wherein the pulmonary veins drain into a common vein that is abnormally incorporated into the SUPERIOR POSTERIOR aspect of the LA creating an EXTRA CHAMBER; an unnecessary fibromuscular membraneous subdivision through the atrial chamber.
Cor TriaTriatum (Classic/Sinister); MC
*CXR: heart is normal sized with changes of chronic interstitial oedema.
MC type of cardiac malposition
Mirror-Image Dextrocardia
*cardiac chambers are completely inverted, and cardiac apex points to the right
Bilateral right-sidedness; severe form; Absent splee, bilateral trilobed lungs; bilateral SVC
Asplenia (IVEMARK Syndrome)
Bilteral left-sidedness; Multiple spleen; bilateral bilobed lungs; interrupted IVC with azygos continuation; biliary atresia;
Polysplenia
*Liver is @ midline and intestinal malrotation occurs