Pansynostosis (Closure of Multiple Sutures) vs. Microcephalia

Pansynostosis (Closure of Multiple Sutures) vs. Microcephalia

Left picture: This 1-year-old boy has a synostosis of multiple sutures. Right picture: The diagnosis in this 3.5-year-old boy is a microcephaly, or more precisely, a microencephaly, considering other clinical findings. In synostosis there are different types of clinical presentation; the presented case reminds of an oxycephaly, in which the skull grows almost exclusively at the site of the anterior fontanel. In case of closure of multiple sutures the head circumference lags successively behind the value in percentiles at birth. In the patient with microcephaly a constitutional microcrania seems unlikely because in the presented child a corresponding familiality is missing, and the neurocranium is to small in comparison to the facial skull. In microencephalia the head circumference is usually too small at birth already; nevertheless, in postnatally acquired cases of microencephaly it may be too small only on follow-up.

The appearance of the neurocranium is very striking in both patients, and the examiner will measure a head circumference too small either in relation to the body length, or in absolute value. Left picture: This 1-year-old boy has a skull which is rather long; there is a tower-shaped prominence anteriorly, with subnormal vault of the front and the back head. Right picture: In this 3.5-year-old child the neurcranium, which is partially covered by hair, is not well developed.