The earliest evidence of true lambdoid craniosynostosis: the case of "Benjamina", a Homo heidelbergensis child.

BACKGROUND: The authors report the morphological and neuroimaging findings of an immature human fossil (Cranium 14) diagnosed with left lambdoid synostosis. DISCUSSION: The skull was recovered at the Sima de los Huesos site in Atapuerca (Burgos, Spain). Since the human fossil remains from this site have been dated to a minimum age of 530,000 years, this skull represents the earliest evidence of craniosynostosis occurring in a hominid. A brief historical review of craniosynostosis and cranial deformation is provided.

In vitro differentiation of human calvarial suture derived cells with and without dexamethasone does not induce in vivo-like expression.

Osteogenic supplements are a requirement for osteoblastic cell differentiation during in vitro culture of human calvarial suture-derived cell populations. We investigated the ability of ascorbic acid and beta-glycerophosphate with and without the addition of dexamethasone to stimulate in vivo-like osteoblastic differentiation. Cells were isolated from unfused and prematurely fused suture tissue from patients with syndromic and non-syndromic craniosynostosis and cultured in each osteogenic medium for varying lengths of time. The effect of media supplementation was investigated with respect to the ability of cells to form mineralised bone nodules and the expression of five osteodifferentiation marker genes (COL1A1, ALP, BSP, OC and RUNX2), and five genes that are differentially expressed during human premature suture fusion (GPC3, RBP4, C1QTNF3, WIF1 and FGF2). Cells from unfused sutures responded more slowly to osteogenic media but formed comparable bone nodules to fused suture-derived cells after 16 days of culture in either osteogenic media. However, gene expression differed between unfused and fused suture-derived cells, as did expression in each osteogenic medium. When compared to expression in the explant tissue of origin, neither medium induced a level or profile of gene expression similar to that seen in vivo. Overall, our results demonstrate that cells from the same suture that are isolated during different stages of morphogenesis in vivo, despite being de-differentiated to a similar level in vitro, respond uniquely and differently to each osteogenic medium. Further, we suggest that neither cell culture medium recapitulates differentiation via activation of the same genetic cascades as occurs in vivo.

Histopathological and biochemical changes in the sutural region in craniosynostosis.

OBJECT: Histopathological observations and biochemical analysis of sutural bones in nine patients with craniosynostosis were compared with control subjects of the same age range. METHODS: Microscopic examination in craniosynostosis showed formation of an active osseous front, with higher osteoblastic activity than in controls. Biochemical analysis revealed higher calcium, phosphorus, alkaline phosphatase, phospholipids and chondroitin sulphate-A contents in sutural bones of the same patients. CONCLUSIONS: The present study systematically establishes a premature increase in osteogenesis in the sutures of craniosynostosis patients.

Diseases of the skull in pre-Columbian South American mummies.

OBJECTIVE: The majority of paleopathological investigations focus on the study of the skull. This is because the skull is the most frequently preserved part of the human body recovered from archaeological excavations. From studying the skull, a variety of information can be obtained regarding the individual, such as sex, age, nutritional status, and other disease processes, if present. METHODS: This study represents the examination of more than 700 human skulls recovered from archaeological excavations from the Andean region of southern Peru and northern Chile and dating back more than 8000 years. RESULTS: A variety of skull abnormalities were encountered. The nonmetric variables of Huschke's foramina and palatine tori were common. Cranial deformation was observed in more than 85% of the cases. There were two cases of sagittal synostosis. Iron deficiency anemia resulting in porotic hyperostosis of the skull was evident in certain cultures. Exostoses of the external auditory canal resulting from chronic otitis was evident only among coastal populations. One skull demonstrated a periostitis consistent with Treponema infection. Trephination was encountered only in the skulls from Peru. Fifty-four cases of skull fractures were observed, half of which showed evidence of healing. Finally, only two cases of neoplastic skull lesions were encountered. CONCLUSION: The study of the human skull alone provides a large amount of information regarding the health and diseases of ancient populations.

Wryneck in the ancient Hawaiians.

A unique cranial asymmetry previously noted in the skeletal remains from Mokapu, O'ahu, Hawai'i, is described. The anomaly involves an indentation of one or both of the occipital condyles and facial and vault asymmetry. This examination of the asymmetry includes a search for other reported occurrences, a detailed description, and a differential diagnosis. A multiple working hypothesis approach is employed. Comparison of the osseous material with the expected clinical pictures in craniosynostosis, Kleippel-Feil syndrome, primary basilar impression, and torticollis results in the most likely explanation of congenital torticollis. A high rate of occurrence of the anomaly (1.8%) is found in the skeletal remains from the Hawaiian Islands, but it has been documented in only two instances outside Hawai'i. A survey of patients seen at The Shriner's Hospital for Crippled Children does not reveal a high rate of occurrence of torticollis in Hawaiians relative to other ethnic groups.