Multiple Wormian bones and their relation with definite pathological conditions in a case of an adult cranium

Abstract

Wormian bones, also known as intrasutural bones, are present as an anatomical variation in healthy individuals. However, a higher than the usual incidence can be an important feature of some congenital pathological conditions. In this study we describe a case of an adult cranium with multiple Wormian bones. The cranium was a single sample obtained from an archaeological excavation in Vinitsa, Northeastern Bulgaria, and probably dated in the Chalcolithic. The Chalcolithic is a period of human history connected with discovering and using of copper. Actually, it is a transitional phase between the Neolithic and the Bronze Age. In this case the Wormian bones are mostly large in size and arranged in a mosaic pattern in several cranial regions. The cranium also shows features such as dolichocrany, a moderate platybasia, a notch in the posterior margin of the foramen magnum, hypoplastic and asymmetrical frontal sinuses, underdeveloped mastoid air cells, hyperostosis cranii interna, moderate frontal bossing, a complete metopic suture, a delayed sutural closure, relatively small facial bones, an early loss of teeth, dental caries and hypoplastic enamel defects on two preserved molars. Differential diagnosis indicates that the combination of all these features shows a link with pathological conditions involving dysplasias with prominent membranous bone involvement and an increased bone density such as cleidocranial dysplasia and pyknodysostosis.

Digital radiomorphometric analysis of the frontal sinus and assessment of the relation between persistent metopic suture and frontal sinus development

“…According to Shapiro and Schorr (), the hypoplasia of the midface blocks one of the major stimuli for the FS pneumatization, i.e., the need to provide a structural bridge between the cranium and the face. A congenital disorders like Hajdu–Cheney syndrome, cleidocranial dysostosis and pyknodysostosis are characterized by such an underdevelopment of the nasomaxillary complex and also feature a preservation of the MS (Castriota‐Scanderbeg & Dallapiccola, ; Nikolova et al, ; Palav, Vernekar, Pereira, & Desai, ). In healthy individuals, the frequent relation between MS preservation and FS underdevelopment is intricate and still unclear.…”

“…Aplasia/hypoplasia of the FS has been associated with Down syndrome, Apert syndrome, Treacher Collins syndrome (Bluestone, ), Hajdu–Cheney syndrome (Canalis & Zanotti, ), cleidocranial dysostosis, pyknodysostosis (Shapiro & Schorr, ), cystic fibrosis (Eggesbø, Søvik, Dølvik, Eiklid & Kolmannskog, ), etc. The persistent metopic suture (MS) is considered to be innocuous, but it is a concomitant finding typical of cleidocranial dysostosis and pyknodysostosis along with underdeveloped sinuses (Castriota‐Scanderbeg & Dallapiccola ; Nikolova, Toneva, Yordanov & Lazarov, ).…”

Sclerosing Bone Dysplasia from 16th Century Sardinia (Italy): A Possible Case of Camurati–Engelmann Disease

“…Sclerosing bone dysplasias are rare genetic diseases whose variety makes the clinical diagnosis challenging; in our case the mature age of the individual, with evident signs of late stage of the disease, helped us identifying the disorder. A few reports of sclerosing bone dysplasias, some of which doubtful and diagnosed in isolated bones (Nielsen & Alexandersen, 1971;Nikolova et al, 2014), have been previously described in paleopathological 839 Sclerosing Dysplasia from 16 th Century Sardinia…”

Variations in the squamous part of the occipital bone in medieval and contemporary cranial series from Bulgaria

“…There are many questions concerning the origin and pattern of fusion of these centres, which cause the numerous variations in this part of the skull. Furthermore, the lambda is a common place for the development of sutural or Wormian bones from their own ossification centres [20,24]. This in turn leads to additional confusion in the classification of variations.…”

“…The lambdoid suture is the most common place for the sutural bones [24]. Their presence is a normal variation, except for the cases when they are larger and numerous and can be an indicator for some congenital pathological conditions [20]. Notwithstanding, the sutural bones could be differentiated from the preinterparietals by their irregular arrangement and shape.…”