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).
- 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.
- 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).
Nuclear Medicine Tests
- None indicated
- None indicated
- 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.