Pediatrics: Skull and Scalp Flashcards
Craniosynostosis
Premature fusion of one or several of the cranial sutures, resulting in a deformed head shape
What are the major cranial sutures?
- Sagittal (separates the bilateral parietal bones)
- Coronal (separates frontal/parietal bones)
- Metopic (splits the frontal bone)
- Lambdoid (separates parietal/occpital bones)
What is the order of fusion of the major cranial sutures?
- Metopic (within 2-3 months of life)
- Coronal
- Lambdoid
- Sagittal
What is the most common form of craniosynostosis?
Sagittal, causing scaphocephaly (long, narrow head)
Metopic synostosis causes…
Trigonocephaly (triangular forehead with eyes close together and underdeveloped ethmoid sinuses)
Sagittal synostosis causes…
Scaphocephaly (long, narrow head)
Coronal synostosis causes…
- Brachycephaly (if bilateral)
- Harlequin eye (if unilateral)
Note: The bilateral form is more rare than the unilateral form.
Lambdoid synostosis causes…
Turricephaly (if bilateral)
Note: This is the least common cranial synostosis.
Complications of metric synostosis
Cognitive disorders (growth restriction of the frontal lobes)
Complications of sagittal synostosis
Usually asymptomatic (normal IQ. no hydrocephalus)
Sagittal synostosis is associated with…
Marfans
Note: Both are tall and skinny (scaphocephaly is a long, narrow head).
Plagiocephaly
Term used to describe flat skull
Note: Anterior plagiocephaly refers to unilateral coronal synostosis and posterior plagiocephaly refers to unilateral lambdoid synostosis. Deformational plegiocephaly is a benign positional molding of the skull not due to a pathologic process.
“quizzical eye” appearance…
Think trigonocephaly due to metric synostosis
“harlequin eye” appearance…
Think brachycephaly due to unilateral coronal synostosis
Positional plagiocephaly
Flattening of the skull due to positioning (e.g. infant sleeps on the same side every night)
Note: This is the most common cause of an abnormal skill shape in an infant.
How can you tell the difference between a true unilateral lambdoid craniosynostosis and positional plagiocephaly?
Unilateral lambdoid craniosynostosis should be present at birth and results in the ipsilateral ear moving posteriorly (towards the flat part)
Positional plagiocephaly develops weeks after birth and results in the ipsilateral ear moving anteriorly (away from the flat part)
If you are suspecting craniosynostosis, what test should you recommend to confirm?
CT with 3D recons
Absence/dysplasia of the greater sphenoid wing…
Think NF-1
Tibial pseudoarthrosis and scoliosis…
Think NF-1
Lateral thoracic meningocele…
Think NF-1
Bone defect in the region of the lambdoid suture OR astern defect…
Think NF-1
Clover leaf skull syndrome (AKA Kleeblattschadel)
Enlargement of the head with a trilobed configuration (resembling a 3-leaf clover) that results from premature synostosis of the coronal and lambdoid sutures (most commonly)
Note: The sagittal suture often also closes.
What is the most significant complication of Kleeblattschadel?
Clover leaf skull syndrome is commonly associated with hydrocephalus
Kleeblattschadel is associated with…
- Thanatophoric dysplasia
- Apert syndrome (severe)
- Crouton syndrome (severe)
Note: This is also called clover leaf skull syndrome.
Apert’s syndrome
- Brachycephaly (usually)
- Fused fingers/toes (syndactyly, often symmetric)
Crouzon’s syndrome
- Brachycephaly (usually)
- Maxillary/mandibular hypoplasia
- Hydrocephalus is common
- Chiari I malformations (70%)
- Associated with patent ductus arteriosus and aortic coarctation
- Short central long bones humerus/femus (rhizomelia)
Note: Crouzon’s C’s (Coronal sutures fused, Can’t Chew, Chiari I, Coarctation, hydroCephalus, Central short bones, Crazy eyes).
On radiographs, you see mild skull marking that are primarily over the posterior skull…
Think convolutional markings (normal gyral impressions on the inner surface of the skull)
On radiographs, you see numerous skull markings along the posterior and anterior skull…
Think “copper beaten” skull due to increased intracranial pressure
Causes of a “copper beaten” skull…
Increased intracranial pressure (e.g. craniosynostosis, obstructive hydrocephalus)
Note: A “copper beaten” skull has numerous skull markings around the entire skull.