Summary
In positional plagiocephaly the skull bone itself is normal – the head is flattened by one-sided lying pressure. The cranial sutures are open and mobile. In craniosynostosis one or more cranial sutures are prematurely fused. The brain cannot grow symmetrically, which can lead to characteristic head shapes, in severe cases raised intracranial pressure and developmental delay. Decisive for the distinction are: ear position, frontal bossing, palpation of the sutures and – for certainty – imaging.
Positional plagiocephaly is very common, with a prevalence of 15–20 % in infants under four months, and has increased since the "back to sleep" campaign of the 1990s. Craniosynostosis is much rarer, occurring in about 1 in 2,500 live births, but can take many forms and is associated, in syndromic variants, with gene mutations.
Comparison
| Feature | Positional plagiocephaly | Craniosynostosis |
|---|---|---|
| Cause | One-sided lying pressure on pliable skull bones; no suture fusion | Premature fusion of one or more cranial sutures |
| Frequency | 15–20 % of infants up to 4 months | approx. 1 : 2,500 live births |
| Ear position | ShiftedEar on the flattened side displaced forward and down | Usually symmetric; no ear advancement |
| Forehead / face | Frontal bossing ipsilateral (same side as flattening), cheek forward | Shape depends on the affected suture: trigonocephaly (metopic), scaphocephaly (sagittal), plagiocephaly (coronal), etc. |
| Palpation of sutures | Sutures open, soft, no palpable ridge | Bony ridgeor absent mobility along the affected suture |
| Head circumference | Normal, symmetric growth | May be compensatorily altered; reduced skull volume with multiple sutures |
| CVAI measurement | Diagnosis and severity by CVAI (> 3.5 % = abnormal, > 6.25 % = severe) | CVAI alone insufficient – 3D CT needed to assess sutures |
| Torticollis | Often associated (30–50 %): muscular torticollis favours a preferred lying position | Rarely associated; no causal link |
| Raised-ICP risk | No riskSkull growth unaffected | PossibleEspecially in multisuture craniosynostosis; ophthalmological check needed |
| Treatment | Physiotherapy, positioning corrections; possibly orthosis (helmet) – KVG covers only physio | Surgical correction (endoscopic or open) in a specialised centre |
| Prognosis | Normalises spontaneously in > 80 % by the 2nd year; early treatment improves the result | Very good with early intervention; if delayed, risk of raised ICP and cognitive impairment |
When to see a doctor?
In most cases an experienced paediatrician or craniofacial surgeon can make the diagnosis clinically. In case of uncertainty or suspected craniosynostosis, the following examinations are used:
| Examination | Yield | When? |
|---|---|---|
| Clinical measurement (CVAI, calliper) | Quantifies asymmetry; follow-up | Initial examination, follow-up |
| 3D photogrammetry | Objective skull-shape measurement without radiation | With unclear findings, treatment monitoring |
| Ultrasound of cranial sutures | Suture patency assessable non-invasively; available in expert centres | First imaging when suture fusion is suspected |
| 3D CT (low-dose protocol) | Gold standard: suture fusion directly demonstrable | Confirmation of craniosynostosis before surgery |
| MRI | Assessment of brain structure, Chiari malformation; no radiation | Complex syndromes, suspected raised ICP |
| Ophthalmology | Papilloedema as a sign of raised intracranial pressure | In craniosynostosis at least yearly |
Treatment pathways
Procedure in Switzerland
In Switzerland the paediatrician is the first point of contact. If craniosynostosis is suspected, referral is made to a specialised centre – in German-speaking Switzerland typically to the Children's Hospital Zurich (Kispi), the Inselspital Bern or the Cantonal Hospital St. Gallen. For French-speaking families the CHUV in Lausanne and the HUG in Geneva are responsible.
A referral is possible informally – a medical letter with the head-circumference curve, photos of the head shape (from above, lateral, frontal) and a description of lying habits considerably speeds up triage.
References
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Further pages on this condition – diagnostics, treatment, cross-cutting topics and research.