PAEDIATRICS 2 Flashcards
What is a Rathke Cleft Cysts?
AKA?
Where is it found?
T1 and T2 features?
Pathognomic sign?
- AKA: Pars intermedia Cysts (if small and asymptomatic)
- non-neoplastic
- sellar or supra sellar epithelium-line cysts arising from the embryologic remant
- well defined non-enhacing midline cyst within the sella arising between the anterior and intermediate lobes of the pituitary.
- 40% are purely intrasellar
- 60% have supra sella extension.
- Purely suprasellar location, although reported, is rare.
- T1 = 50% hyper (secondary to high protein content), 50% hypo
- T2 = 70% are hyperintense, 30% are iso or hypointense.
- T1 C+ = no contrast enhancement of the cyst is seen.
- 75% of cases a small non-enhancning intracystic nodule can be identified which is virtually pathognomoic of a Rathke cleft cyst.
What is the dx?
What are 5 causes?
Hydraencephaly
Hydranencephaly is a rare encephalopathy that occurs in-utero. It is characterised by destruction of the cerebral hemispheres which are transformed into a membranous sac containing cerebrospinal fluid and the remnants of cortex and white matter 1. Porencephaly is considered a less severe degree of the same pathology 10
Five aetiologies have been described:
- infarction: bilateral occlusion of the supraclinoid segment of the internal carotid arteries or of the middle cerebral arteries 3
- leukomalacia: an extreme form of leukomalacia formed by confluence of multiple cystic cavities 4
- diffuse hypoxic-ischaemic brain necrosis: fetal hypoxia due to maternal exposure to carbon monoxide or butane gas may result in massive tissue necrosis with cavitation and resorption of necrotised tissue 1
- infection: necrotising vasculitis or local destruction of the brain tissue secondary to intrauterine infection, e.g. congenital toxoplasmosis, cytomegalovirus, and herpes simplex(HSV) infections 1,8
- thromboplastic material from a deceased co-twin monochorionic twins have presented with a variety of cerebral lesions. lesions in the recipient twin result from emboli or thromboplastic material originating from the macerated co-twin 4
Case Discussion
There is marked enlargement of fluid spaces with little if any overlying cerebral parenchyma except for the posterior fossa and para medial location along the falx.
The posterior fossa structures are inferiorly displaced and the fourth ventricle is not well seen. Higher cuts demonstrate marked cerebral destruction of both cerebral hemispheres predominately in the distribution mid cerebral arteries with minimal residual tissue identified both anteriorly and posteriorly. The anterior fontanelle is bulging.
This case demonstrates typical CT and ultrasound appearances of hydranencephaly, which is in almost all cases not compatible with significant post natal survival.
https://radiopaedia.org/cases/hydranencephaly-1?lang=us
What is this?
Uterus
What are US prepubertal features of the Uterus
The prepubertal uterus has a tubular configuration (AP cervix equal to AP fundus) or sometimes a spade shape (AP cervix larger than AP fundus).
The endometrium is normally not apparent; however, high-frequency transducers can demonstrate the central lining.
The length is 2.5–4 cm; the thickness does not exceed 10 mm.
https: //pubs.rsna.org/doi/pdf/10.1148/radiographics.21.6.g01nv041393
* Figure 2. Prepubertal uterus. (a) Longitudinal US scan obtained in a 5-year-old girl shows a tubular uterus; the anteroposterior diameter is 6 mm. (b) Longitudinal US scan obtained in a 6-year-old girl shows the endometrial lin- ing as a thin echogenic line (arrow).*
- LUL colapse causes in kids
- FB
- endobronchia lesion -> carcinoid
- mucous plug
Three facts about Crossed Renal Ectopia
- 1/1000
- M:F 2:1
- 90% are fused.
What is the incidence of Congenital diaphragmatic hernia (CDH)?
What is the mortality rate?
What is the most reliable predictor of post natal survival?
What are the clinical symptoms?
What are 3 associated anomalies?
- Incidence: 1/2000-3000 births.
- Mortality rate: isolated hernias is 60%.
- Higher when other abnormalities are present.
- Most reliable predictor of postnatal survival is absence of liver herniation.
- Respiratory distress occurs in neonatal period.
- Associated abnormalities include:
- Pulmonary Hypoplasia
- CNS abnormalities.
- NTDs
- Anencephaly.
What is this?
What sequence should you ask for?
What is it associated with clinically?
Band Heterotopia
Ask for:
- Inversion Recovery Sequence
- A/W seizures
Case courtesy of Dr Ian Craven, Radiopaedia.org, rID: 78613
What is the modality of choice for DDH and why?
- USS is the modality of choice prior to the ossification of the proximal femoral epiphysis.
- Once there is a significant ossificaiton then an xray is required.
- USS is the test of choice in <6 months as the proximal femoral epiphysis has not yet signficantlly ossified.
- Real time dynamic examinatio allowing the stability of the hip to the assessed with stress views.
3 types of Pulmonary Hypoplasia
- agenesis:
- complete absence of one or both lungs.
- agenesis:
- Aplasia:
- abscence of lung except for a rudimentary bronchus that ends in a blind pouch.
- Aplasia:
- -Hypoplasia:
- decrease in the number and size of airways and alveoli: hypoplastic PA.
What is the rule for assessing Splenomegally in paediatric patients?
Max splenic size (cm)
- 1/2 Pts age +6
What is the Hilgenreiner line?
- Drawn horizontally through the superior aspect of both triradiate cartilages.
- It should be horizontal but is mainly used as a reference for the Perkin line
- Measurment of the acetabular angle
The Perkins line and Shenton arc on radiography. Anteroposterior radiograph in a 2-year-old girl with left developmental dysplasia of the hip. The Perkins line is drawn perpendicular to the Hilgenreiner line (H line) and intersects the lateral acetabular rim. The Shenton arc is formed by the medial cortex of the femoral neck and the inferior cortex of the superior pubic ramus (dotted lines). The normal right hip shows the femoral head confined to the inferomedial quadrant and a continuous Shenton arc. The abnormal left hip shows the dislocated femoral head within the superolateral quadrant and a discontinuous Shenton arc
https://www.researchgate.net/figure/The-Perkins-line-and-Shenton-arc-on-radiography-Anteroposterior-radiograph-in-a_fig10_337009090
What is Laryngomalacia?
Clinical symptoms?
When does it occur?
How do you diagnose it?
- Common cause of stridor in the first year of life.
- Immature laryngeal cartilage leads to supraglottic collapse during inspiration.
- Stridor improves with activity and is relieved by propositions or neck extension.
- Self-limited course.
- diagnosis is established by fluoroscopy where there is laryngeal collapse with inspiration.
Etiology: congenital cartilage abnormality in larynx
Imaging: dynamic partial supraglottic collapse of the larynx during breathing
https://pediatricimaging.org/diseases/laryngomalacia/
Which has normal Pulmonary Blood flow on CXR?
- VSD
- TOF
- AP window
- PDA
- Coarctation
(Paeds tute 10.9.20)
Co-arctation
- PDA > L-> R Shunt -> Plethoric
- VSD > L-> R Shunt -> Plethoric
- TOF -> oligaemic
What is a Craniopharyngioma?
- intro
- relatively benign neoplasms that typically arise in the sellar/suprasellar region.
- 1-5% of primary brain tumors and can occur anywhere along the infundibulum, from the floor of the 3rd ventricle to the pituitary gland.
- bimodal distribution 5-15years (adamantinomatous)
- 40 years (both papillary and adamantinomatous)
- no gender predilection.
- Subtypes
- adamantinomatous (more common)
- papillary
- mixed/transitional (imaging and prognosis similar to adamantinomatous).
- Ddx
- Intracranial teratoma
- presence of fat is helpful but requires fat sat sequences or CT to confirm
- Pituitary macroadenoma
- can look very similar
- usually has intrasellar epicenter with pituitary fossa enlargement rather than the suprasellar epicenter
- don’t usually calcify
- Rathke cleft cyst
- no solid or enhancing component
- calcification is rare
- unilocular
- the majority are completely or mostly intrasellar.
- https://www.facebook.com/watch/?v=557576101663321
- Intracranial teratoma
Wolffian Duct
- anlage for vas deference, seminal vesicles, epididymis
- guides ureteral migration to the bladder.
- induces kidney development and ascent
- induces Mullerian duct development in females.
Name the following vascular rings.
- Double aortic arch
- Innominate artery compression
- Left arch with aberrant right subclavian artery. or right arch with abberant left subclavian artery.
- Aberrant lefft pulmonary artery (pulmonary sling)
LCH Extraskeleta manifestations
Pulmonary Involvement
alveolar disease (exudate of histiocytes)
interstitial pattern (upper lobe pred)
CNS
meningeal involvement
pituitary
RES organs
Liver, spleen, LNS
12 Paediatric Soft tissue tumours
- Rhabdomyosarcoma
- Ewings Sarcoma
- Synovial Sarcoma
- MPNST
- Liposarcoma
- Kaposi Sarcoma
- Undifferentiated pleomorphic sarcoma
- Leiomyosarcoma
- Epitheliod Sarcoma
- GIST
- Angiosarcoma
- Dermatofibrosarcoma Proteuberans
CHARGE SYNDROME
Which gene?
What are the main characteristics?
CHARGE syndrome is a phenotype associated with CHD7 gene mutation originally defined by a constellation of congenital anomalies:
- C: coloboma
- H: heart defects
- A: atresia choanae
- R: retarded growth and development
- G: genital hypoplasia
- E: ear abnormalities and/or deafness
According to updated diagnostic criteria, the most defining features are the 4 C’s:
- coloboma
- choanal atresia
- cranial nerve anomalies (especially olfactory pathway absence)
- characteristic ear anomalies (especially semicircular canal dysplasia/aplasia)
- Epidemiology
- The incidence is 1-12 per 100,000 births 6.
- Clinical presentation
- CHARGE syndrome is usually suspected at birth once multiple congenital abnormalities are identified.
- Diagnosis
- The diagnosis of CHARGE syndrome can be made on clinical grounds 6,11:
- definite CHARGE syndrome: 4 major characteristics or 3 major plus 3 minor characteristics
- possible/probable CHARGE syndrome: one-to-two major characteristics and several minor characteristics
- Major criteria
- coloboma (80%): ranges from defect of iris, retina, choroid, or disc, to microphthalmia or anophthalmia
- choanal atresia/stenosis (45%) or cleft palate (25-50%)
- cranial nerve anomaly/dysfunction
- olfactory (90%): hyposmia/anosmia
- facial (40%): facial palsy
- vestibulocochlear (95-100%): sensorineural deafness
- glossopharyngeal or vagal (60-80%): velopharyngeal incoordination for suck/swallow
- characteristic ear anomalies (some or all of the following) (90%)
- abnormal auricle: short and wide (lop/cup shaped), absent lobule, truncated helix, prominent antihelix
- ossicular malformations
- Mondini malformation
- absent/hypoplastic semicircular canals
- Mondini malformation
- Minor criteria
- urogenital abnormalities
- kidney
- duplex kidney
- renal hypoplasia/solitary kidney
- penis
- hypospadias
- penile agenesis
- scrotum/testes
- bifid scrotum
- cryptorchidism
- vaginal atresia
- uterine atresia
- congenital heart disease
- tetralogy of Fallot
- atrioventricular canal defect
- double outlet right ventricle
- short stature
- cleft palate +/- lip
- esophageal atresia / tracheo-esophageal fistula (~15%) 2
- characteristic facies
- developmental delay
- urogenital abnormalities
- Pathology
- CHARGE syndrome is thought to occur due to a disturbance in embryonic differentiation ~35th to 45th day of gestation.
CHARGE syndrome. Axial CT image in a 6-day-old boy (a) shows bilateral bony and membranous choanal atresia with a thickened vomer and medial deviation of the lateral nasal walls at the level of the choanae (black arrows). There are secretions layering within the nasal cavities (*). Axial T2-weighted MRI at 4 days of age (same patient) (b) shows bilateral colobomas (arrowheads). Coronal T2weighted MRI (c) shows that the olfactory apparatus is absent on the right, but intact on the left (curved arrow). Axial CISS (constructive interference in steady state) image (c) shows bilateral absent semicircular canals and hypoplastic vestibules (white arrows). There is also bilateral cochlear nerve aperture and internal auditory canal stenosis
https://www.researchgate.net/figure/CHARGE-syndrome-Axial-CT-image-in-a-6-day-old-boy-a-shows-bilateral-bony-and_fig8_270650182
Causes of nephrocalcinosis
Cortical: “COAG”
- Renal cortical necrosis
- Oxalosis
- Alport syndrome
- (chronic) glomerulonephritis
Medullary:
- deposition of calcium salts in the medulla of the kidney.
- Causes “HAM HOP”
- hyperparathyroidism
- renal tubular Acidosis
- Medullary sponge kidney
- Hyper or hypo thyroidism
- Oxalosis
- papillary necrosis
- Hypervitaminosis D.
- Milk alkali syndrome
- Sarcoid
-year-old male presents with hematuria. An MRI scan was performed. Axial and coronal T2-weighted images are shown.
There is a polypoid mass in the bladder. The grape-like appearance is characteristic of the botryoid type of rhabdomyosarcoma. Rhabdomyosarcomas are the most common malignant bladder masses in the pediatric population.
Paragangliomas of the bladder may have a similar appearance with a macrolobulated appearance and T2-hyperintense signal, but they are more often seen in patients ages 30 to 60. Hemangiomas are often seen in the setting of other vascular malformations and are similar in appearance to hemangiomas seen elsewhere. Cystitis glandularis may present as nodules on a background of diffuse bladder wall thickening. Bladder neurofibromas are rare and may demonstrate a target appearance on T2-weighted imaging, with central low signal and peripheral high signal.
Epiglottitis
- bacterial infection of the upper airway by H influenza
- 3-6 years old
- Rtx with prophylactic intubation for 24-48 hours and antibiotics
- fever, dysphasia, drooling, sore throat
- X-ray: thickened aryepiglottic folds, lateral view: thickened epiglottis, subglottic narrowing because of edema
- 25% indistinguishable from croup on AP view.
- Distension of hypopharynx.
Tracheal stenosis
Three types:
- diffuse Hypoplasia (30%)
- Focal ring like stenosis (50%)
- Funnel like stenosis (20%)
Rathke Cleft Cysts
- AKA: Pars intermedia Cysts (if small and asymptomatic)
- non-neoplastic
- sellar or supra sellar epithelium-line cysts arising from the embryologic remants
- well defined non-enhacing midline cyst within the sella arising between the anterior and intermediate lobes of the pituitary. 40% are purely intrasellar and 60% have supra sella extension. Purely suprasellar location, although reported, is rare.
- T1 = 50% hyper (secondary to high protein content), 50% hypo
- T2 = 70% are hyperintense, 30% are iso or hypointense.
- T1 C+ = nocontrast enhancement of the cyst is seen.
- 75% of cases a small non-enhacning intracystic nodule can be identified which is virtually pathognomoic of a rathke cleft cyst.
Types of CPAM/CCAM
- Congenital Pulmonary airway Malformation or Congenital Cystic adenomatoid malformation
-
Definition:
- multi-cystic masses of segmental lung tissue with abnormal bronchial proliferation.
- Part of the spectrum of bronchopulmonary foregut malformations.
- Type 0: rare and fatal
- Type 1: Most common, large Cysts (2-10cm) unilateral
- Type 2: Common, multiple small cysts (0.5-2cm), associated with other congenital abnormalities.
- Type 3: Uncommon, large, solid
- Type 4: uncommon, thin walled, often multifocal, high association with malignancy, specifically pleuropulmonary blastoma.
- Type 1 and 4 are difficult to differentiate and carry the risk of malignancy (especially type 4).
- Treatment is resection.
Common mediastinal tumours 2/3 MIDDLE
- Adenopathy
- leukaemia,
- lymphoma,
- tuberculosis
- BFM
- Vascular malformations
Uterus
The
Osteochondroma DDX
- Hands:
- bizarre parosteal osteochondromatous proliferation (BPOP)
- arises from the bone cortex and lacks medullary continuity.
- Case courtesy of Dr Matt Skalski, Radiopaedia.org, rID: 30785
- Humerus:
- supracondylar spur: projects towards the elbow joint.
- Malunited fracture.
CHARGE SYNDROME
- Coloboma
- Heart defects
- choanal Atresia/Cranial nerve defects (facial nerve palsy), Dysphagia.
- Retardation of growth and development
- Gential +/- urinary abnormalities
- Ear abnormalities
Dx:
- Definate CHARGE:
- 4 major characteristics
- 3 major + 3 minors.
- POSSIBLE CHARGE:
- 1 or 2 major characteristics and several minor characteristics
- https://radiopaedia.org/articles/charge-syndrome
Common orbital lesions
- OSA
- 3% of children
- Morbidities
- poor performance in school
- ADD
- Excessive daytime sleepiness
- FTT.
- Secondary to enlargement of the adenoids and palatine tonsils.
- Palatine tonsils can be evaluated clinically
- imaging is limited to a lateral Xray of the airway to evaluate the adenoids which appear as a soft tissue mass in the posterior nasopharynx.
- criteria for enlargement
- >12mm and a convex outward anterior border.
- complete obstruction of the posterior nasopharynx.
Abusive head trauma
Subglottic stenosis
- Fixed narrowing at the level of the cricoid. - Failure of laryngeal recanalisation in utero
Common mediastinal tumours 2/3 MIDDLE
Adenopathy (leukaemia, lymphoma, tuberculosis) BFM Vascular malformations
VSD
- left to right shunt 1/400 births
- 40% of CHD
- more common in neonates
- right atrial enlargement
- LVH
- Plethora
- Cant see AP window secondary to PAH
- cx
- aneurysm to PA
- eisenmegners -> early oligaemia to apices
- A/w T21, T18, T13
- Holt oram syndrome
- TOF
- TA
- coarctation
- AR
- PS
Causes of nephrocalcinosis
Cortical: “COAG”
- Renal cortical necrosis
- Oxalosis
- Alport syndrome
- (chronic) glomerulonephritis
Medullary:
- deposition of calcium salts in the medulla of the kidney.
- Causes “HAM HOP”
- hyperparathyroidism
- renal tubular Acidosis
- Medullary sponge kidney
- Hyper or hypo thyroidism
- Oxalosis
- papillary necrosis
- Hypervitaminosis D.
- Milk alkali syndrome
- Sarcoid
Uterus
The
Ddx High bowel obstruction term neonate
- Gastric atresia
- single bubble
- duodenal atresia
- Double bubble
- UGIS is performed in those patients with a double bubble
and distal gas as these findings will inform the diagnosis.
- duodenal stenosis (including annular pancreas, web, jejunal atresia)
- Jenunal atresisa
- tripple bubble
- malrotation with volvulus
- The presence of distal gas, alongside a
history of bilious vomiting, is very important as it may
represent intestinal malrotation with volvulus, which constitutes
an emergency, requiring confirmation through an
urgent UGIS (or other appropriate diagnostic test). - UGIS is performed in those patients with a double bubble
and distal gas as these findings will inform the diagnosis. - there is a “corkscrew” of the duodenum and jejunum (Fig 4),
then the diagnosis of malrotation with volvulus is made.17
Identification of a “corkscrew” requires notification of the
on-call surgeon immediately as this patient requires urgent
surgery.17 - If there is no corkscrew, but the duodenojejunal (DJ)
flexure is malpositioned (inferior to the first part of the
duodenum or to the right of the spine) then malrotation
without volvulus is diagnosed2 (Fig 5). - An incomplete narrowing
of the duodenum with a “windsock” expansion of
the duodenum indicates a duodenal web18 (Fig 6).
- The presence of distal gas, alongside a
- Hypertrophic pylorus (usually slighlty older infants)
Questions to ask self when looking at AXR
- Is there distal gas? no - complete, yes - incomplete (duodenal web/stenosis/recent malrotation with volvulus)
- single, double or tripple bubble?
Germinal Matrix Haemorrage
- GM is a fetal structure that is a stem source for NEUROBLASTS.
- Tupically involutes by term but is still present in prem infants within the Caudothalamic groove.
- Highly vascular and subject to hemorrhage with fluctuation in cerebra blood pressure.
- Hx most commonly occues in premature infants during the first days after birth.
- Complications include
- destruction of the precursor cells within the geminal matrix
- hydrocephalus
- hemorrhagic inffection of the surrounding periventricular tissues
A 17-year-old male was playing football and fell on his right shoulder and had pain that persisted for several days
There is an expansile lesion in the proximal humerus at the metadiaphysis. No pathologic fracture is seen. There are no findings of osteoid or chondroid matrix. The most likely diagnosis is an aneurysmal bone cyst, which is typically found in patients younger than 20 years of age in the long bones at the metadiaphysis. As in this case, radiographs will demonstrate a sharply defined expansile lesion with sclerotic margins.
This MRI demonstrates a T1-bright, multicystic structure in the proximal humerus with multiple fluid-fluid levels and no enhancing soft-tissue components, confirming the diagnosis of an aneurysmal bone cyst. cHONDROBLASTOM, gIANT CELL TUMOUR, OSTEO BLASTOMA AND FIBROUIS DYSPLASIA can be seen with aneurysmal bone cysts except intraosseous lipomas. Intraosseous lipoma does not have ABC formation
Corner fractures Classic metaphyseal lesions
classical metaphyseal lesions
-year-old male presents with hematuria. An MRI scan was performed. Axial and coronal T2-weighted images are shown.
There is a polypoid mass in the bladder. The grape-like appearance is characteristic of the botryoid type of rhabdomyosarcoma. Rhabdomyosarcomas are the most common malignant bladder masses in the pediatric population.
Paragangliomas of the bladder may have a similar appearance with a macrolobulated appearance and T2-hyperintense signal, but they are more often seen in patients ages 30 to 60. Hemangiomas are often seen in the setting of other vascular malformations and are similar in appearance to hemangiomas seen elsewhere. Cystitis glandularis may present as nodules on a background of diffuse bladder wall thickening. Bladder neurofibromas are rare and may demonstrate a target appearance on T2-weighted imaging, with central low signal and peripheral high signal.
DDH Beta angle
- formed by the vertical cortex of the ilium and the triangular labral fibrocartilages (echogenic triangle).
- The normal value is less than 77 degrees, but is only useful in assessing immature hips when combined with the alpha angle.
- There is a great deal of variability in the beta angle (much more than the alpha angle), and as such, it is not universally used.
- In some instances, the beta angle is necessary to determine the subtype of hip dysplasia (e.g. type Ia vs. Ib, or type IIc vs. type D - see the sonographic classification of developmental dysplasia of the hip (DDH)). The cut off beta angle depends on type 3.
Renal findings in TS
- Aniogmyolipomas AMLs are an abnormal collection of vessels smooth muscle and fat cells seen in 55 to 75% if TS patients
- Classically AMLs can be distinguished from other enhancing renal leison by macroscopic fat.
- Additional renal manifestations include renal cysts renal cell carcinoma, renal oncocytomas and renal failure.
What are the signs of VSD on CXR?
What are the differentials of a Widened Joint Space in paediatric patients?
Widened Joint Space ( Fig. 11.98 )
Widened joint spaces in pediatric patients are most commonly seen in hip joint or shoulders; other joints have strong capsules. Causes of widened joint space include:
- Joint effusion
- Septic arthritis
- Hemarthrosis (intraarticular fracture, hemophiliac)
- Transient toxic synovitis (viral)
- JRA
- Synovial thickening without articular cartilage destruction
- JRA
- Hemophiliac arthropathy
What are the main characteristics of the different Chiari Malformations?
What is an association of each type of chiari?
The SHORT Overview of Chiari Malformations
- Chiari I =
- downward displacement of cerebellar tonsils below foramen magnum (>5 mm);
- unrelated to Chiari II malformation
- Chiari II =
- abnormal neurulation leads to a small posterior fossa,
- caudal displacement of brainstem and
- herniation of tonsils and vermis through the foramen magnum;
- myelomeningocele
- Chiari III =
- encephalocele and
- Chiari II findings (rare)
- https://www.drawittoknowit.com/course/pathology/neurological-pathologies/developmental-disorders/1418/hindbrain-malformations
THE LONG
Chiari 1
- Intermittent compression of the brainstem:
- Nerve palsies
- Atypical facial pain
- Respiratory depression
- Long tract signs
- Associations
- Syringohydromyelia, 50%;
- weakness of hands, arms, loss of tendon reflexes
- Hydrocephalus, 25%
- Basilar invagination, 30%
- Klippel-Feil anomaly: fusion of 2 or more cervical vertebrae, 10%
- Atlantooccipital fusion, 5%
- Syringohydromyelia, 50%;
- Imaging Features
- Tonsillar herniation (ectopia is 3–5 mm, herniation is >5 mm) is age dependent.
- Syringohydromyelia
- No brain anomalies
CHIARI 2
- Most common in newborns
- Associations
- Myelomeningocele, 90%
- Obstructive hydrocephalus, 90%
- Dysgenesis of corpus callosum
- Syringohydromyelia, 50%
- Abnormal cortical gyration
- Chiari II is not associated with Klippel-Feil anomaly or Chiari I.
- Imaging Features ( Fig. 6.73 )
- Posterior fossa
- Small posterior fossa
- Cerebellar vermis herniated through foramen (verminal peg)
- Upward herniation of cerebellum through widened incisure (towering cerebellum)
- Cerebellum wraps around pons (heart shape).
- Low, widened tentorium
- Obliterated CPA cistern and cisterna magna
- Nonvisualization or very small fourth ventricle
- Supratentorium ( Fig. 6.74 )
- Hypoplastic or fenestrated falx causes interdigitation of gyri (gyral interlocking).
- Small, crowded gyri (stenogyria), 50%
- Hydrocephalus almost always present before shunting.
- Bat wing configuration of frontal horns (caused by impressions by caudate nucleus), enlarged atria and occipital horns (colpocephaly)
- Small, biconcave third ventricle (hourglass shape because of large massa intermedia)
- Beaked tectum
- Posterior fossa
- Osseous abnormalities
- Lückenschädel skull (present at birth, disappears later)
- Scalloped clivus and petrous ridge (pressure effect)
- Enlarged foramen magnum
- Spinal cord
- Myelomeningocele, 90%
- Cervicomedullary kink at foramen magnum (pressure effect)
- Syringohydromyelia and diastematomyelia
USD CXR
What is this??
What are the different types?
What can be useful on CT to determine the different types?
Choanal Atresia
- Membranous or bony obstruction of the nasal passage in the newborn.
- If bilateral, respiratory distress at birth as newborns are obligate nasal breathers.
- Failure to pass small catheter through nasal cavity.
CT: findings of enlarged vomer and medial bowing of the lateral walls of the nasal cavity are useful to distinguish bony atresia from membranous.
Axial Ct widening of the vomer (long arrow), bowing of the posteromedial maxilla (short arrow), and narrowing of the choana anterior to the pterygoid.
https://www.researchgate.net/figure/Axial-Ct-widening-of-the-vomer-long-arrow-bowing-of-the-posteromedial-maxilla-short_fig7_51505685
Intro
Incidence
Arises from?
Clinical findings?
% metastatic at presentation?
- Most common abdominal malignancy in the newborn;
- rare tumor (incidence 1 : 30,000).
- Arises in neural crest tissue (adrenal medulla, sympathetic neural system, Zuckerkandl).
- Age: 2 years, better prognosis <1 year
- Clinical Findings
- Elevated vanillymandelic acid (VMA) and homovanillic acid (HVA)
- May cause paraneoplastic syndrome
- Radiographic Features
- Solid tumor
- Hyperechoic by US
- Calcification in 85%
- Readily extends across midline
- Frequently encases vessels
- 65% are metastatic at initial presentation;
- common extensions include:
- Bone
- Neural foramina (evaluate)
- Lymph nodes
- Liver, lung (uncommon)
- Case courtesy of Rad_doc, Radiopaedia.org, rID: 47939
What is this?
What is the underlying pathology?
What is the most common location?
- Results from abnormal budding of the tracheobronchial tree.
- Cysts contain respiratory epithelium.
- Location
- mediastinum (85%
- Posterior>
- middle >
- anterior)
- mediastinum (85%
- lung (15%)
- Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 6313
Neuro tumours
- Gliomas
- astrocytomas
- ependymomas
- Medulloblastoma
- Retinoblastoma
- heritable form
- Non-heritable (unilateral)
- There is a lobulated mass within the right globe that is T1-hyperintense and heterogeneously T2-hypointense relative to vitreous fluid, and which displays restricted diffusion and contrast enhancement. There is retinal detachment with associated hemorrhage, resulting in a fluid/fluid level in the right globe seen on the T2-weighted images.
Retinoblastoma is the most common intraocular childhood malignancy, with a median presentation of 12 months. It can be sporadic (approximately 60% of cases), which requires two spontaneous mutations of the retinoblastoma protein tumor suppressor gene (RB), or it can be familial, due to a single spontaneous mutation in combination with an inherited germline mutation.
Retinoblastomas appear as mixed calcified and noncalcified soft-tissue intraocular masses. CT and ultrasound are good for depicting intralesional calcifications. On MRI, they will appear as a T1-hyperintense, T2-hypointense mass relative to vitreous fluid. Retinoblastomas will restrict on diffusion imaging given that they have a high cellular content as they fall within the class of small blue round cell tumors. The contralateral globe, pineal region, and suprasellar regions need to be carefully evaluated for the presence of additional sites of disease.
Coats disease is characterized by progressive retinal detachment by exudative sub retinal collections and is not typically associated with enhancement, restricted diffusion or calcification.
A coloboma is congenital malformation characterize by failure of closure of the choroidal fissure and is seen as a small globe with a posterior defect with vitreous herniation.
Astrocytic hamartomas are benign retinal tumors associated with tuberous sclerosis.
Retinopathy of prematurity is found in premature infants and seen as vascular proliferation with asymmetrically small globes.
- DNET
- dysembryoplastic neuroepithelial tumour
A 4-year-old female with a history of seizures presents with altered mental status. A noncontrast-enhanced CT scan of the head was performed. Axial image is shown
CT demonstrates extensive periventricular calcifications. Periventricular calcifications are most characteristic of CMV, which is the most common TORCH infection. Polymicrogyria also may occur.
Toxoplasmosis calcifications of the basal ganglia with hydrocephalus.
Rubella demonstrates ischemia with high T2 signal on MRI.
HSV may result in hemorrhagic infarct, with resulting encephalomalacia.
HIV also results in atrophy, predominantly in a frontal distribution.
