Peds -- Neuro

Front 1

1. Is there a gender predilection for craniosynostosis?
2. What is the most common suture to be involved?
3. What are the causes of primary and secondary craniosynostosis?
4. Which craniofacial syndromes are associated with craniosynostosis?

Back 1

1. Yes. It is 4x more common in males.
2. Sagittal suture resulting in dolichocephaly (AKA scaphocephaly).
3.
Primary craniosynostosis:
- failure of dural development, or
- ischemic injury to the growth plate.
Secondary craniosynostosis:
- rickets, hypophosphatasia, anemia, or decreased intracranial pressure
4.
Consider a syndromic cause of craniosynostosis when you have fusion of bilateral coronal and/or lambdoid sutures.
- Apert syndrome: "Mitten hand" due to syndactyly and fusion of the toes.
- Carpenter syndrome:
- Crouzon disease:
**All 3 disorders lead to midfacial hypoplasia with prominent bulging eyes, however, only Apert is associated with syndactyly.

Front 2

What is trigonocephaly?

Back 2

Abnormal “wedge shaped” or “triangular” appearance to the frontal bone of the skull
Premature closure of the metopic suture

Front 3

What is scaphocephaly?

Back 3

AKA dolichocephaly occurs as a result of premature closure of the sagittal suture.
Look for a long, narrow skull that looks "boat shaped" in appearance.

Front 4

1. What is brachycephaly?
2. What is plagiocephaly?
3. What are Harlequin eyes?

Back 4

1. Refers to premature close of both coronal or lambdoid sutures.
2. Plagiocephaly refers to isolated unilateral closure of coronal or lamboid suture producing an asymmetrically shaped skull.
3. Harlequin eye results from distorted orbital shape due to unilateral or bilateral coronal suture synostosis.

Front 5

What is oxycephaly?

Back 5

Oxycephaly (turricephaly): vertical development of the skull from premature closure of coronal and sagittal sutures

Front 6

1. What is Kleeblattschadel or cloverleaf skull?
2. What is it associated with?

Back 6

1. Premature synostosis of multiple sutures (coronal, lambdoid, sagittal)
2. Associated with thanatophoric dwarfism

Front 7

1. What is periventricular leukomalacia (PVL)?
2. What is the most common location of periventricular leukomalacia?
3. What are the imaging findings?

Back 7

1. Periventricular leukomalacia (PVL) represents white-matter ischemia that leads to coagulation necrosis, phagocytosis, and eventual cyst formation. It may be a sequelae of previous intraparenchymal hemorrhage (usually 2/2 venous infarction).
2. Peritrigonal white matter and near the foramen of Monroe.
3.
- Peritrigonal white matter hypoattenuation or T2 hyperintensity.
- Petechial hemorrhage seen on MRI.
- Cysts of varying size in acute/subacute phase which eventually resolve resulting in ventricular enlargement.
- Decreased thickness of the white matter and corpus callosum. Cortical sulci may abut the ventricular wall.
- On US, there is increased periventricular echogenicity with cyst formation.

Front 8

Describe the grading of intracranial hemorrahge in neonates.

Back 8

- Grade I: subependymal hemorrhage only
- Grade II: Intraventricular hemorrhage without hydrocephalus
- Grade III : Intraventricular hemorrhage with hydrocephalus.
**Blood within the ventricle will often lead to ventriculitis demonstrated by thickening of the echogenic subependyma.
**Progressive hydrocephalus requires shunting.
Grade IV: intraparenchymal hemorrhage 2/2 venous infarction. Usually occurs in the frontal or parietal lobes. The result of venous hemorrhagic infarction is formation of cystic porencephaly in periventricular WM.
**Evolving hemorrhage will lose its echogenic appearance and eventually become echolucent. Cysts found at birth suggest fetal hemorrhage.

Front 9

1. What is the most common TORCH infection?
2. What are the imaging findings of CMV infection?
3. What are the imaging findings of toxoplasmosis infection?

Back 9

1. CMV
2. Imaging findings of CMV include,
- Periventricular calcifications
- Lenticulostriate vasculopathy: branching curvilinear hyperechogenicity/calcifications in the thalamus
- Gray matter migrational disorders: polymicrogyria and lissencephaly
- Cerebellar hypogenesis
3. Imaging findings of toxoplasmosis infection include,
- SCATTERED multifocal calcifications involving the basal ganglia + periventricular WM + cerebral cortex
- Hydrocephalus
- Encephalomalacia

Front 10

DDX of lenticulostriate vasculopathy (mineralizing vasculopathy)

Back 10

Top 3:
- TORCH infections
- Maternal cocaine abuse
- Idiopathic

Less common:
- Hypoxic-Ischemic encephalopathy
- Maternal SLE
- Twin/twin transfusion
- Fetal ETOH syndrome
- Trisomy 13

Front 11

1. What is the ddx of HYPERtelorism?
2. What is the ddx of HYPOtelorism?

Back 11

1. HYPERTELORISM:
- Cleidocranial dysostosis
- Mucopolysaccharidosis (Hunter or Hurler syndrome)
- Thalassemia
- Cephalocele
2. HYPOTELORISM:
- Holoprosencephaly
- Microcephaly
- Craniosynostosis: trigonocephaly and scaphocephaly.

Front 12

Lytic lesion in skull

Back 12

LCH
- Bevelled edges
- Likes to affect the mastoid portion of the temporal bone
- Look for floating tooth sign in the jaw.
EPIDERMOID CYST
- 2nd most common cause after LCH.
- 2/2 abnormal deposition of epithelial rests within the diploic space during development.
NEOPLASM
- Leukemia, Ewing sarcoma, and metastatic NB
INFECTION
LEPTOMENINGEAL CYST
- "growing skull fracture"
- caused by CSF pulsations from disruption of dura.