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Am J Med Genet A. 2005 Aug 1;136A(4):327-42.

Malformations of the axial skeleton in the museum Vrolik: II: craniosynostoses and suture-related conditions.

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1
Department of Anatomy and Embryology, Academic Medical Center, University of Amsterdam, The Netherlands. R.J.Oostra@amc.uva.nl

Abstract

The Museum Vrolik collection of anatomical specimens in Amsterdam, The Netherlands, comprises over 5,000 specimens of human and animal anatomy, embryology, pathology, and congenital anomalies. Recently, we rediagnosed a subset of the collection comprising dried infantile, juvenile, and adult human skulls with congenital and acquired conditions. On external examination and additional radiography, we found 58 skulls with craniosynostosis (CS) involving one or more sutures and 40 skulls with a presumed suture related condition. Most of these were part of the material collected and described by Louis Bolk (1866-1930). Analysis of his observations suggests that skull deformation because of premature suture closure depends not only on the identity of the sutures involved but also on the timing and progression of their closure and the extent of their involvement. Moreover, premature closure of the sagittal suture after 3-6 years of age appeared to be much more common than expected because it is not accompanied by skull deformation. Many of the skulls with single-suture CS were microcephalic, which may be the cause of the premature synostosis. By contrast, microcephaly may be a resulting phenomenon in multi-suture CS. We noticed that the quotient between height of the cranial vault (vertex-porion distance) and head circumference, multiplied by 100, was 26 or higher only in those CS cases with multi-suture involvement. We therefore consider this parameter, which we named "acrocephalic index", to be an indicator of multi-suture involvement in individual CS cases. In two adult skulls, the skull had a quadrangular shape, which we assumed to be correlated to the presence of an unusually interdigitated open metopic suture. We propose to name this anomaly: tetragonocephaly. Another presumed suture-related condition, bathrocephaly, was found concomitantly with basilar invagination in several cases. We hypothesize that the chronically raised intracranial pressure in these cases caused the still open lambdoidal sutures to distend and the occipital bone to protrude.

PMID:
15937945
DOI:
10.1002/ajmg.a.30769
[Indexed for MEDLINE]
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