The ISPN Guide to Pediatric Neurosurgery

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Evaluation of Nonsyndromic Craniosynostosis in Children

Examination

Characteristic head shapes

  • Scaphocephaly – sagittal synostosis: Scaphocephaly is a specific term used for the head shape associated with sagittal craniosynostosis. “Dolichocephaly” is a general term used for the long narrow head shape that may be positional in nature. The skull has a long AP diameter with a small bitemporal/biparietal width. There may be frontal bossing and occipital cupping. Less common are bathrocephaly (localized suture synostosis) and clinocephaly (severe scaphocephaly with vertex indentation).
  • Anterior plagiocephaly – coronal synostosis: Anterior plagiocephaly results in a flattened forehead over the involved suture with a foreshortened orbit, a prominent forehead on the opposite side, and a forward projection of the ipsilateral ear.
  • Trigonocephaly – metopic synostosis: Trigonocephaly describes a triangular forehead, decreased intracanthal distance, recession of the orbits, and compensatory biparietal flaring.
  • Posterior plagiocephaly – lambdoid synostosis: Posterior plagiocephaly results in a rhomboid shape to skull (as opposed to parallelogram shape of positional plagiocephaly). It is characterized by a posteriorly placed ipsilateral ear with bulging, inferior placed mastoid and fullness of the contralateral parietal bone. This type of craniosynostosis must be differentiated from posterior positional plagiocephaly (64).

 

Posterior positional plagiocephaly (left) and lambdoid craniosynostosis (right): In posterior positional plagiocephaly, note ear placement anterior and frontal prominence ipsilateral. In lambdoid craniosynostosis, note ear placement posterior, mastoid protuberance, and frontal prominence contralateral.
   
Positional plagiocephaly: Note the flattened left occipital and ipsilateral forward projection of the forehead that characterizes deformational plagiocephaly.

 

Laboratory Tests

  • Primary craniosynostosis: No definite lab work required.
  • Secondary craniosynostosis: A genetics and endocrinology evaluation is needed to assess the metabolic abnormality that has caused the misshapen head.

Radiologic Tests

  • Single suture craniosynostosis: Radiographs are unnecessary for a typical child.
  • Atypical head shapes: This may require a CT head with fine cuts and three-dimensional reconstructions. Remember to evaluate the axial fine cut bone windows as well as the three-dimensional reconstructions.
  • SPECT: SPECT scans have been performed in research studies and may indicate a difference in perfusion under the fused suture (15, 39).
CT scan for sagittal synostosis: A suture ridge is noted at the sagittal suture.

 

 

Nuclear Medicine Tests

  • None indicated

Electrodiagnostic Tests

  • None indicated

Neuropsychological Tests

  • Neurodevelopmental testing can be considered: Recent neurocognitive studies suggest developmental delays in children with isolated single suture fusions.  Close follow-up in craniofacial clinic or with neurodevelopmental testing may be in order ( 36, 43, 45).

Correlation of Tests

  • Appearance key: In most cases the child’s appearance will lead to the diagnosis, and a series of tests will not be needed.