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We present a case of infantile myofibromatosis manifest as a choroid plexus mass followed by spontaneous regression. Infantile myofibromatosis is a common juvenile fibrous disorder occurring in infancy and early childhood. Intracranial involvement in infantile myofibromatosis is rare. It generally occurs in the dura with calvarial invasion and secondary brain compression. [ABSTRACT FROM AUTHOR]

The aim of the present study was to test the osteogenic effects of BMP-2 (bone morphogenetic protein-2) gene transfer in BMSCs (bone-marrow stromal cells) and rabbit calvarial bone defects. The pBMP-2-cDNA3.1 plasmid was constructed by subcloning hBMP-2 (human BMP-2) cDNA into the plasmid pcDNA3.1. BMSCs were transfected with a pBMP-2-cDNA3.1–Lipofect-amine™ complex. Transfected cells were observed for localization of the BMP-2 coding plasmid. Also, the level of BMP-2 in the culture medium of transfected cells was measured. The culture medium was collected and we tested whether this medium could induce non-transfected BMSCs to express ALP (alkaline phosphatase) and osteocalcin. The pBMP-2-cDNA3.1 complexes were incorporated into the collagen scaffold and the plasmid-loaded collagen scaffolds were then grafted into rabbit calvarial defects. After 2 weeks, granulation tissue at the grafted site was obtained and mRNA of BMP-2 was examined via RT (reverse transcriptase)–PCR. After 4 and 8 weeks, the animals were killed and the calvarial tissue was excised. After specimen preparation, optical microscopical examination was performed to evaluate bone formation. The results show that transfected cells were able to incorporate the BMP-2 gene into their nuclei. Also, the level of expressed and secreted BMP-2 was significantly higher in transfected cells than in untransfected cells (P<0.01). The retrieved culture medium could induce the expression of ALP and osteocalcin in non-transfected BMSCs. hBMP-2 mRNA was detected at the granulation tissue of experimental animals, but not in control animals after 2 weeks. At both 4 and 8 weeks, experimental groups showed significantly more newly formed bone area than the control group (P<0.01). Therefore pBMP-2-cDNA3.1 gene delivery could induce BMSCs into osteoblastic phenotype cells and enhance bone regeneration in rabbit calvarial bone defects. [ABSTRACT FROM AUTHOR]

Osterix (Osx) is a zinc-finger-containing transcription factor that is expressed in osteoblasts of all endochondral and membranous bones. In Osx null mice, osteoblast differentiation is impaired, and bone formation is absent. We hypothesized that overexpression of Osx in bone marrow–derived mesenchymal stem cells (BMSCs) would enhance osteogenic differentiation during bone regeneration in vivo. Overexpression of Osx in mouse BMSCs was achieved using retroviral infection together with a green fluorescent protein (GFP) vector to monitor transduction efficiency and determine the source of regenerative cells in implantation studies. Bone regeneration in vivowas evaluated by implanting BMSCs overexpressing Osx into 4-mm calvarial bone defects in adult mice using type I collagen sponge as a carrier. New bone formation in the defects was quantified using radiological and histological procedures 5 weeks after implantation. The results showed that implantation of Osx-transduced BMSCs resulted in 85 healing of calvarial bone defects as detected using radiological analyses. Histological examination of the implants demonstrated that the Osx-transduced group exhibited amounts of newly formed bone that was five times as high as in a group transduced with the empty vector. Immunohistochemistry for GFP showed positive immunoreaction localized to areas of newly engineered bone in the Osx-transduced group. Immunohistochemistry with antibodies against the extracellular matrix protein bone sialoprotein resulted in strong staining in areas of new bone formation. In addition, the clonal BMSCs showed an osteogenic potential similar to that of primary cultures of BMSCs, suggesting the usefulness of this model in bone tissue engineering. These results indicate that ex vivogene therapy of Osx is a useful therapeutic approach in regenerating adult bone tissue. [ABSTRACT FROM AUTHOR]

Cases of pathologically changed bone might constitute a diagnostic pitfall and frequently need histological methods to be etiologically properly evaluated. With micro-computed tomography (μCT), a new epoch of 2D and 3D imaging has been launched. We evaluated the diagnostic investigation of this analytical method versus well established histological investigations of historical human bone. Pathological changes due to various etiologies (infectious, traumatic, endocrinological, neoplasia) observed in autopsy-based macerated human skulls (Galler Collection, Natural History Museum Basel, Switzerland) were investigated by μCT and compared with histological thin ground sections using polarized light. Micro-CT images visualize the architecture of the bone with high spatial resolution without preparation or destruction of the sample in the area to be sectioned. Changes in the bone surfaces as well as alterations of the diploe can be assessed. However, morphological patterns caused by reactive response, such as typical arrangements of collagen fibers, can only be visualized by the microscopic investigation of thin ground sections using polarized light. A great advantage of μCT is the high number of slices obtained so that spatial differences within the areas of the specimen become visible. Micro-CT is a valuable tool for the diagnosis of vestiges of skull bone diseases. Its advantages over histology are the fast, automated image acquisition and the fact that the specimen is not completely destroyed. Only excision of the area to be scanned is necessary, if the specimen is too large to be scanned as a whole. Further, the 3D visualization of the micro-architecture allows an easy orientation within the sample, for example, for the choice of the location of the histological slices. However, the need to differentiate woven from lamellar bone still makes histology an indispensable method. [ABSTRACT FROM AUTHOR]

Summary: Introduction: Noggin is an antagonist of bone morphogenetic proteins (BMP)-2, -4 and -7. Little data are available regarding its clinical utility. Two hypotheses were put forward: firstly, that spontaneous regeneration of calvarial defects with noggin protein would result in diminished bone volume when compared with calvarial defects not so treated. Secondly, that centrifugal cranial expansion would remain undisturbed whether noggin was applied or not. Material and methods: A unilateral defect of the frontal and parietal bones (2×4cm) was generated by excising the right coronal suture in 2-month-old minipigs (n=10) and in group 1 (n=5) no further intervention was undertaken. In the second group (n=5), a collagen type I tissue fleece and noggin protein (1.05mg/ml) were applied. After 4 months the coronal suture regions of frontal sides were examined in each animal by computed tomography and non-decalcified histology. Results: Bony gaps of equivalent size remained in animals of both groups. The differences in bone volumes of the experimental sides of group 1 were not statistically significantly different (p=0.117) when compared with those of group 2. A significant difference in the bone volumes of the experimental versus control (unoperated) sides was found in both group 1 (p=0.043) and group 2 (p=0.043). Internal skull diameters increased by 16.4% in both groups but the physiological centrifugal cranial expansion remained undisturbed. Bone densities of the experimental and control sides of groups 1 and 2 were not statistically significantly different (both p>0.05). Conclusions: The first hypothesis was contradicted: the quantity and quality of spontaneous bone regenerates was not altered by application of noggin protein. The second hypothesis was confirmed: no disruption of subsequent cranial development was seen. It may be that a single application of noggin protein in this study was insufficient. However, it may well be suggested that the continuous supplementation of noggin, for example by adenoviral noggin gene transfer may significantly reduce the quantity of spontaneous bone regeneration in a similar experiment. [Copyright &y& Elsevier]

Hyperostosis frontalis interna (HFI) is a disease characterized by excess bone growth on the internal lamina of the frontal bone and, occasionally, other cranial bones. Although the disease is fairly common in modern populations, its etiology is poorly understood. Hyperostosis frontalis interna has been identified in antiquity, primarily in the Old World, but with a much lower frequency than in modern groups. The purpose of the present study is to report multiple cases of HFI at Pueblo Bonito (Chaco Canyon, New Mexico). Twelve out of 37 adults with observable frontal bones exhibited HFI, ranging from mild to severe, including 11 females and one male. This is the first published case report of HFI in archaeological remains from the New World having a frequency comparable with modern groups. Most archaeological cases of HFI are isolated, so comparative data for multiple cases at one site are rare. The results of this study emphasize the importance of looking for HFI in archaeological remains, although it is rarely observed. Possible genetic and environmental factors for the high frequency of HFI at Chaco Canyon are considered, but additional research is needed to discover the etiology and to better understand why HFI sometimes occurs at modern frequencies in ancient populations. [ABSTRACT FROM AUTHOR]

Abstract: Two cases of thickening of the internal tables of the frontal bones (hyperostosis frontalis interna, (HFI)) have been examined. These were two female skeletons from the 16th century Dominican Church in Raciborz (Southwest Poland). The similarity of their morphological and metrical traits indicates that they could be related, and suggests that HFI is likely to have a genetic base. These two skeletons are the subject of an analysis which may possibly throw some new light on the controversial and continually disputed nature of this illness. [Copyright &y& Elsevier]

Two bullmastiffs with calvarial hyperostosis syndrome are described and are the first documented examples in females. The clinical and radiologic features were similar to those previously reported in males. Magnetic resonance (MR) imaging findings have not previously been reported. One dog underwent MR imaging and abnormalities included thickening of the frontal bones with loss of normal fat signal and changes in the overlying soft tissues. In one of the dogs, long bone changes were seen in the femora and resembled those seen with craniomandibular osteopathy. [ABSTRACT FROM AUTHOR]

Comparative morphological studies of the earliest human skeletons of the New World have shown that, whereas late prehistoric, recent, and present Native Americans tend to exhibit a cranial morphology similar to late and modern Northern Asians (short and wide neurocrania; high, orthognatic and broad faces; and relatively high and narrow orbits and noses), the earliest South Americans tend to be more similar to present Australians, Melanesians, and Sub-Saharan Africans (narrow and long neurocrania; prognatic, low faces; and relatively low and broad orbits and noses). However, most of the previous studies of early American human remains were based on small cranial samples. Herein we compare the largest sample of early American skulls ever studied (81 skulls of the Lagoa Santa region) with worldwide data sets representing global morphological variation in humans, through three different multivariate analyses. The results obtained from all multivariate analyses confirm a close morphological affinity between South- American Paleoindians and extant Australo-Melanesians groups, supporting the hypothesis that two distinct biological populations could have colonized the New World in the Pleistocene/Holocene transition. [ABSTRACT FROM AUTHOR]

We report two 71-year-old female monozygotic twins presenting with advanced hyperostosis frontalis interna, obesity, shortness and cognitive impairment. They both have suffered from generalized seizures since their early adulthood. Moreover, the patients showed some additional conditions only occurring in one individual or the other such as migraine, marked recurrent depressive disorder or polyarthrosis. The symptoms common to both twins appear to correspond to the Morgagni-Stewart-Morel syndrome and indicate a genetic basis of this disorder as these features occur in genetically identical patients. Copyright © 2005 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Presents a study which aims define the incidence and associations of hypertosis frontalis interna with other pathological conditions as revealed by medico legal authorities. Determination on the incidence of HFI as revealed by medico-legal autopsies; Description on the associated symptoms of HFI; Importance of HFI in medico-legal indentification.

A 77 year old woman who presented with an incarcerated hernia of Morgagni was successfully treated without complications. A Medline search (1996 to date) along with cross referencing was done to quantify the number of acute presentations in adults compared to children. Different investigating modalities-for example, lateral chest and abdominal radiography, contrast studies or, in difficult cases, computed tomography or magnetic resonance imaging-can be used to diagnose hernia of Morgagni. The favoured method of repair-laparotomy or laparoscopy-is also discussed. A total of 47 case reports on children and 93 case reports on adults were found. Fourteen percent of children (seven out of 47) presented acutely compared with 12% of adults (12 out of 93). Repair at laparotomy was the method of choice but if uncertain, laparoscopy would be a useful diagnostic tool before attempted repair. Laparoscopic repair was favoured in adults especially in non-acute cases. [ABSTRACT FROM AUTHOR]

The purpose of this study was to relate the pathological and imaging features of dural enhancement and meningeal sign (“dural tail”) on contrast-enhanced T1-weighted magnetic resonance (MR) images from patients with primary calvarial lesions as well to assess the accuracy of MR imaging in predicting dural invasion. Thirty-two calvarial tumors studied with contrast-enhanced MR imaging and histopathological examination of the dural specimens were reviewed. Sixteen patients presented dural enhancement, eight with tumor invasion. Tumoral invasion of the dura was observed in one case without enhancement. Malignant lesions showed enhanced dura more commonly than benign lesions (P=0.02). Nodular and discontinuous dural enhancement was statistically associated with dural invasion (P=0.05). Dural tail did not show a specific pathological association. Meningeal enhancement is a nonspecific reaction to calvarial lesions unless nodular and discontinuous. False-negative and -positive cases of dural invasion imply some limitation of contrast-enhanced MR imaging in predicting dural invasion by calvarial neoplasms. [ABSTRACT FROM AUTHOR]

Abstract: Hyperostosis frontalis interna is a restricted bilateral thickening of the frontal endocranial surface, which is frequently found in postmenopausal females today. Surprisingly, this condition had a higher male prevalence in its rare archaeological records. This is again highlighted by the oldest known male European hyperostosis frontalis interna case in an adult Celtic from 100 BC presented here. This unique specimen supports earlier suggestions of the possible microevolution of human endocrine regulation, e.g. by sex steroids, and its patho-anatomical impact. [Copyright &y& Elsevier]

Background Various surgical techniques for the treatment of craniosynostosis using distraction devices have been described over the last few years and we have applied these procedures in seven patients with varying types of craniosynostosis. The aim of this report is to clarify the advantages and disadvantages of these surgical methods and to discuss current concepts for the surgical strategy in the treatment of craniosynostosis. Material and methods From January 2001 to March 2003, 25 patients with craniosynostosis were examined. Among them, 7 patients, 5 with Apert syndrome, 1 with Crouzon disease, and 1 with multiple-synostosis, underwent surgical treatment using the distraction method with internal distraction devices, according to our treatment strategy for craniosynostosis. All patients underwent preoperative and postoperative evaluations, which included the patient’s neurological state, developmental quotient (DQ), and three-dimensional CT (3D-CT). Results The timing of the procedures undertaken was between the ages of 1 year 5 months and 12 years 6 months (mean age 4 years 11 months). Five patients had received previous treatment and this procedure was used as a secondary operation. Postoperative distraction distances varied from 7 to 20.5 mm (mean distraction distance: 14 mm). Satisfactory cranial volume expansion and aesthetically pleasing morphological states were achieved in all cases. Regarding complications, one patient required re-operation because of dislocation of the device and skin erosion caused by infection around the penetrated wound. Finally, in a second patient a distortion of the device occurred, but no re-operation was needed. Conclusion The advantage of the distraction method is its applicability for Toddler or Elder Children Calvarial Reconstruction to correct cosmetic and functional problems. One disadvantage is the difficulty in using it for Infantile Calvarial Normalization because of thin calvarial bones and the necessity for re-operation to remove the device, which may result in it becoming a “fixation procedure,” essentially contraindicated for the fast-developing brain and calvarias. However, the efficacy of this procedure is that the many advantages outweigh the disadvantages as sufficient calvarial expansion and good results using the distraction method, especially in toddler and elder children age groups, can be achieved. [ABSTRACT FROM AUTHOR]

B7-H3, a member of the B7 family of the Ig superfamily proteins, is expressed on the surface of the antigen-presenting cells and down-regulates T cell functions by engaging an unknown counter receptor on T cells. Although B7-H3 is ubiquitously expressed, its potential nonimmune functions have not been addressed. We found that B7-H3 is highly expressed in developing bones during embryogenesis and that its expression increases as osteob last precursor cells differentiate into mature osteoblasts. In vitro bone formation by osteoblastic cells was inhibited when B7-H3 function was interrupted by the soluble recombinant protein B7-H3-Fc. Analysis of calvarial cells derived from neonatal B7-H3 knockout (KO) mice revealed normal numbers of osteoblast precursor cells possessing a normal proliferative capacity. However, the B7-H3- deficient calvarial cells exhibited impaired osteogenic differentiation, resulting in decreased mineralized bone formation in vitro. These results suggest that B7-H3 is required for the later phase of osteoblast differentiation. Although B7-H3 KO mice had no gross skeletal abnormalities, they displayed a lower bone mineral density in cortical (but not trabecular) bones compared with WT controls. Consistent with the reduced bone mineral density, the femurs of B7-H3 KO mice were more susceptible to bone fracture compared with those of WT mice. Taken together, these results indicate that B7-H3 and its unknown counterreceptor play a positive regulatory role in bone formation. In addition, our findings identified B7-H3 as another molecule that has a dual role in the bone-immune interface. [ABSTRACT FROM AUTHOR]

The nose is the central part of the face, and constitutes the most prominent projection in facial geometry. This report presents five cases that sustained a chemical burn injury with associated facial mutilation resulting from contact with strong acids. The chemical burn affected the nasal architecture after inflicting a burn injury to the face. Applying bone deriving from a split skull procedure for the nasal projection restoration and the augmentation of the dorsal nose is a feasible undertaking, and the overall result appears satisfactory.A retrospective survey of cases admitted to our clinic from January 1999 to December 2001, inclusively was undertaken and is described below. Split calvarial bone graft procedure for the nasal tip projection reconstruction was performed for five patients, all of whom had sustained chemical burns following assault by strong acid. The disfiguration of the nasal anatomical structure was due to healing from deep burn wounds. The tip became blunt and less protruberant following the arising of cicatricial tightness of the surrounding tissue. Strength and resistance to scar contraction are the first considerations for such implantation when attempting to correct the nasal tip projection. The five female patients sustained a severe chemical burn which involved a surface area ranging from 25 to 60% of total body surface area. The facial mutilation was noted simultaneously with the determination of the extent of the burning injury. A severe burn scar is the typical sequel following a deep chemical burn. Nasal tip projection was restored and a nasal dorsum augmentation procedure with a split calvarial bone graft under an “open” method was used. This particular surgical procedure was able to be used in order to improve the nasal tip projection and resist surrounding scar contracture. The three-dimensional surface structure of the face became more prominent subsequent to the administration of this procedure. [Copyright &y& Elsevier]

We report three elderly patients with hyperostosis cranii. Patient 1 had two episodes of unconsciousness; Patient 2, headache; and Patient 3, dementia. On the basis of Moore's classification using skull films, Patients 1 and 2 showed hyperostosis frontoparietalis and Patient 3 had hyperostosis frontalis interna. Electroencephalography showed transient generalized spike and slow wave complexes over the frontal lobes in Patient 1. Magnetic resonance images showed frontal lobes compressed by the thickness of the frontal bones in all patients and the thickness of the parietal bones in Patients 1 and 2. Since the findings in the present cases and those in the literature suggest that hyperostosis cranii could show unexpected neuropsychiatric symptoms, hyperostosis cranii should be checked in elderly patients whose presenting symptoms include epilepsy, dementia, psychiatric disease, headache and so on. Magnetic resonance images should be helpful in examining the relationship between clinical symptoms and the deformation of the brain by the skull. [ABSTRACT FROM AUTHOR]

Objective: The recommended treatment for solitary calvarial eosinophilic granuloma (EG) is surgical resection by curettage, craniectomy or craniotomy. The purpose of this study was to describe the spontaneous resolution of calvarial EG and discuss ‘observation only’ as an option in the management of this condition. Methods: A retrospective review was performed of children with calvarial EG seen at a tertiary care children’s hospital. Of a total of 17 such patients, four were managed without any intervention and formed the basis of this report. Results: In all four patients treated by ‘observation only’, there was a tender calvarial mass which enlarged rapidly over a few days and then resolved slowly over many weeks. The diagnosis of EG was based on the findings of plain radiographs and/or computed tomography. All masses had resolved by 8 weeks, and the underlying cranial defect filled in spontaneously in all cases. There was no recurrence at follow-up, which ranged from 2 months to 7 years. Conclusions: ‘Observation only’ should be considered as a viable option in the management of children with solitary calvarial masses that have the radiographic features of EG. Consideration should be given to delaying surgical intervention to allow time to see if spontaneous resolution occurs.Copyright © 2003 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

An analysis, visualization and modelling of the dynamics of craniofacial growth is presented, which is based on a Karhunen-Loève decomposition of time-discrete landmark data describing the craniofacial skeleton and its growth. This allows for a clear and suggestive depiction of craniofacial growth patterns and their dynamical behaviour, which is necessary for medical diagnosis as well as for orthodontic treatment. For modelling the dynamics, a medically motivated approach based on a dynamical system with general and individual parameters is used to obtain the underlying general growth laws. The parameters are identified by a least squares method for fitting the model to the growth data which consist of a rat calvarial data set and data of a human growth study. This allows for a quantitative description of the dynamics of size and shape changes as well as a qualitative prediction of growth processes. [ABSTRACT FROM AUTHOR]

Background: The cholesterol-lowering drug simvastatin has been shown to stimulate murine calvarial bone growth after multiple injections. The purpose of this study was to test if similar bone stimulation could be induced by 2 single-dose drug delivery systems appropriate to periodontal therapy.Methods: ICR Swiss mice were treated with the following protocols: 1) injection of methylcellulose gel alone, subcutaneously over the calvarium (INJ-GEL; n = 8); 2) injection of gel with simvastatin (INJ-SIM; 2.2 mg, n = 16); 3) polylactide membrane (PLA) containing gel alone implanted over calvarium (MEM-GEL; n = 10); 4) implanted PLA membrane containing gel and simvastatin (MEM-SIM; n = 10); and 5) untreated mice (n = 12). Animals were sacrificed after 22 or 44 days, calvaria decalcified and stained with hematoxylin and eosin, and images digitized and measured for bone thickness and area. Data were compared using analysis of variance.Results: INJ-SIM stimulated a 53% (P = 0.02) increase at the thickest point of calvarial bone, while MEM-SIM caused a highly significant (P < or = 0.0005) increase in bone thickness (159% to 172%) and bone area (144% to 180%) compared to gel controls. Simvastatin gels caused soft tissue inflammation, which appeared to be related to bone increases. If INJ-SIM animals showing leakage of gel and/or no inflammation were excluded from analysis, INJ-SIM resulted in more bone (58% to 83%) than gel controls. An insignificant amount of SIM-stimulated bone was lost over the long term (44 days).Conclusions: A single, high dose of simvastatin gel can stimulate murine cranial bone apposition, particularly when delivered under an occlusive membrane. Both approaches should be investigated further for possible development for periodontal therapy. [ABSTRACT FROM AUTHOR]

Abstract Objective: The objective of this study was to evaluate healing patterns of critical-size calvarial bone defects treated according to principles of guided bone regeneration using micro-CT scan analysis. Specifically, the contribution of bone, periosteum and dura mater to the amount and mineralization of newly formed bone was evaluated. Material and Methods: Surgically induced, critical-size calvarial bone defects in 48 adult male Wistar rats received the following: an occlusive expanded polytetrafluoroethylene (ePTFE) membrane at the exo- and endocranial aspect (OO; n = 12); an occlusive membrane at the exocranial and a perforated membrane at the endocranial aspect (OP; n = 12); a perforated membrane at the exocranial and an occlusive membrane at the endocranial aspect (PO; n = 12); and a perforated membrane at the exo- and endocranial aspect (PP; n = 12). The animals were euthanized at 4 weeks for quantitative analysis of bone volume fraction and mineralization in the region of interest (ROI) as well as in the external, middle and central area of the defect using micro-CT. Results: Bone volume fraction ranged from 31.4% (OP) to 24.5% (PP). No differences were found among the groups. Bone volume fraction and mineralization in the middle area were significantly greater in group OP than in group PP, and in the central area in group OO and PO than in group PP. Conclusions: The results of this study suggest that use of occlusive ePTFE membranes enhances bone formation and maturation in the calvarial skeleton. When occlusion of endo- and exocranial tissues was compromised by membrane perforation, impaired bone formation and mineralization were observed. [ABSTRACT FROM AUTHOR]

To evaluate possible cause-effect relationships between hyperostosis frontalis interna and cognitive dysfunction, we performed a neurophysiological (event-related potentials, ERPs) and neuropsychological study in a case of Morgagni-Stewart-Morel (MSM) syndrome associated with frontal lobe compression. Neuropsychological evaluation evidenced selective impairment of executive function. Visual and auditory oddball ERPs revealed delayed P300 latency and reduced auditory P300 amplitude with multi-peaked morphology. ERP abnormalities and cognitive dysfunction could be due to the frontal bone-cortex conflict documented by neuroradiological investigations. [ABSTRACT FROM AUTHOR]

The article presents a case study of a nine-month spayed female mixed-breed dog with history of hard swelling dorsal to the left frontal bone. It mentions that the dog was referred to the Iowa State University Llyod Veterinary Medical Center. It also offers information on the Idiopathic calvarial hyperostosis.

Presents information on a study on hyperostosis frontalis interna. Materials and methods; Results; Discussion; Conclusions.

Addresses previously undescribed pathological endocranial hyperostosis in fossil hominids. Nature and distribution of hyperostosis; Probable causes; Differential diagnosis; Implications; Descriptions of pathological hyperostosis; Existence and frequency of hyperostosis calvariae internal in fossil hominids.

A 74 year old patient, EW, with dorsolateral frontal cortical compression due to hyperostosis frontalis interna, in the absence of the Morgagni or Stewart-Morel syndromes, is described. In addition to conventional neuropsychological measures EW was administered one nonspatial and two spatial self ordered working memory tasks, as well as a standard measure of fluid intelligence or g. She showed impaired performance on all three self ordered working memory tasks compared with a normal control group of 10 subjects matched for age, education, sex, and IQ. By contrast, her performance on the fluid intelligence test was comparable with that of the controls. It is concluded that the compression of dorsolateral frontal cortex accompanying hyperostosis frontalis interna may produce selective cognitive impairment. [ABSTRACT FROM AUTHOR]

The cAMP-responsive element (CRE) binding protein/activating transcription factor (CREB/ATF) family plays a major role in the expression of skeletal-specific genes and skeletal tissue development. We analyzed the changes of the amount, degree of phosphorylation and binding activity of the CREB/ATF family in the course of development of the murine calvarial osteoblastic cell line MC3T3-E1 as an in vitro model system of bone formation. The amount of CREB in the whole-cell extract detectable by Western blot analysis was high through all stages of development and maximal in the proliferation stage. The degree of phosphorylation estimated with anti-phosphorylated CREB antibody changed greatly and reached high levels in the proliferation stage and early mineralization stage. The ratio of phosphorylated CREB to total CREB in the CREB-CRE complex was also examined by gel shift assay. Although the binding to the consensus/CRE probe reached almost equally high levels in the proliferation stage and early mineralization stage, the relative level of phosphorylated CREB in the CREB-CRE complex was different in these two stages. In the early mineralization stage, most CREB bound to consensus/CRE was phosphorylated, while both phosphorylated and unphosphorylated CREB were bound to consensus/CRE in the proliferation stage. ATF-1 was also detected as a minor component bound to the consensus/CRE probe. The alteration of the binding of CREB to consensus/ CRE over the course of osteoblast development supports the hypothesis that CREB may regulate the expression of genes defining the developmental sequence of MC3T3-E1 cells. [ABSTRACT FROM AUTHOR]

Forty-six mentally ill women with radiologically verified hyperostosis frontalis interna (HFI) were compared with 46 matched mentally ill women without HFI in respect of mental morbidity among the full siblings of the two groups. It was found that mental morbidity was lower among the siblings on mentally ill women with HFI than among those of similar mentally ill women without HFI. A reasonable interpretation of these findings is that the combination mental illness-HFI is more often exogenously determined than other forms of mental illness. [ABSTRACT FROM AUTHOR]

We describe a rare case of vascularised orbital roof and calvarial erosions with an associated venous malformation. In the absence of infection, malignancy, trauma and eosinophillic granuloma, the closest previously described entity is vanishing bone disease. Computed tomography ( CT), MRI, catheter angiography and pathology were all important in the diagnostic workup to enable surgical planning for biopsy and reconstruction. Ongoing CT and MRI follow-up imaging will determine future treatment planning. [ABSTRACT FROM AUTHOR]

The article describes the case of a 62-year-old man with a large Morgagni hernia. Topics discussed include the complex medical history of the patient, the results of the computed tomography (CT) scan and positron emission tomography scan done on the patient and the upper midline laparotomy performed on the patient. Also mentioned is the identification of hemorrhagic right middle lobe via thoracoscopy.

The authors discuss aspects of surgical procedure for the fixation of nasofrontal suture exostosis in horses. They explain the benefits of perioperative antimicrobial prophylaxis in equine surgeries for incidence of infection. The authors suggest that antimicrobial prophylaxis may be considered during implants to prevent the development of infection from devastating consequences of unsterile cavity and microbial contamination in the paranasal sinusis.

Background: Cranioplasty aims to reconstruct skull defects from fractures, decompressive craniectomies, tumors, and congenital anomalies in a cosmetically acceptable manner. We present a technique in methyl methacrylate cranioplasty that gives excellent cosmetic results by maintaining patient's calvarial curvature. Method: Cranioplasty material is placed into a plastic bag and packed inside the defect. Wire mesh cut larger than the defect is held in position to take the exact skull curvature. Once solid, the implant is fixed in position using titanium plates and mini-screws. Conclusion: This is a simple, inexpensive method of achieving the most cosmetically desired cranioplasty results. [ABSTRACT FROM AUTHOR]

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Presents a study which examined the occurrence of hyperphosphatasemia in patients with hyperostosis frontalis interna. Materials and methods; Results; Discussion.