Russell-Silver syndrome (RSS) is a congenital disease characterized by short stature due to growth hormone deficiency, physical asymmetry, inverted triangular face, micrognathia, prominent forehead, and hypodontia. This case report presents a prosthetic management of a 6-year-old patient with Russell-Silver syndrome treated with overdentures on the maxilla and the mandible using the remaining primary teeth. Subsequent and comprehensive dental management considering the growth and development of a young patient will be necessary.
Anodontia ; Child ; Denture, Overlay ; Forehead ; Growth and Development ; Growth Hormone ; Humans ; Mandible ; Maxilla ; Silver-Russell Syndrome* ; Tooth, Deciduous

Surgical correction is central to current team-approached cleft treatment. Cleft surgeons are always concerned about the impact of their surgical maneuver on the growth of the maxilla. Hypoplastic maxilla, concaved mid-face and deformed dental arch have constantly been reported after cleft treatments. It is very hard to completely circumvent these postoperative complications by current surgical protocols. In this paper, we discussed the factors that inhibit the maxillofacial growth on cleft patients. These factors included pre-surgical intervention, the timing of cleft palate and alveolae repair, surgical design and treatment protocol. Also, we made a review about the influence on the maxillary growth in un-operated cleft patients. On the basis of previous researches, we can conclude that most of scholars express identity of views in these aspects: early palatoplasty lead to maxilla growth inhibition in all dimensions; secondary alveolar bone graft had no influence on maxilla sagittal growth; cleft lip repair inhibited maxilla sagittal length in patients with cleft lip and palate; Veau's pushback palatoplasty and Langenbeck's palatoplasty with relaxing incisions were most detrimental to growth; Furlow palatoplasty showed little detrimental effect on maxilla growth; timing of hard palate closure, instead of the sequence of hard or soft palate repair, determined the postoperative growth. Still, scholars hold controversial viewpoints in some issues, for example, un-operated clefts have normal growth potential or not, pre-surgical intervention and pharyngoplasty inhibited maxillofacial growth or not.
Adolescent ; Child ; Child, Preschool ; Cleft Lip ; surgery ; Cleft Palate ; surgery ; Face ; Humans ; Infant ; Maxilla ; growth & development ; Preoperative Period

OBJECTIVEThis study aims to investigate the temporal pattern of expression of vascular endothelial growth factor (VEGF) and new bone formation during midpalatal suture expansion osteogenesis for rapid maxillary expansion.

METHODSA total of44 New Zealand white rabbits were randomly assigned to 11 groups, namely, five experimental groups, five control groups, and one control 0 group. A Haas appliance was used for the rapid expansion of the midpalatal suture; rapid expansion was set for 2 weeks and fixed for 4 weeks. The tissue of the maxillary midpalatal suture was harvested on the day of installing rapid expansion (control 0 group), on weeks 1 and 2 for rapid expansion, and on weeks 1, 2, and 4 for fixed (experimental group and control group, respectively). The immunohistochemical method was used to detect distribution and expression of VEGF, and new bone formation was observed with periodic acid-Schiff.

RESULTSHigher VEGF expressions are observed after midpalatal suture distraction osteogenesis. Positive staining for VEGF is mainly noted in the vascular endothelial cells, and the active osteoblasts are at the edge of a newborn trabecular bone. A weak VEGF expression is detected among cells in the control group. The VEGF expression of the experimental group is higher than that of the control group in rapid expansion weeks 1 and 2 and in fixed weeks 1 and 2. The expression of VEGF in the experimental group increases significantly and peaks at fixed week 1, and then gradually decreases. The amount of newly formed bone in the experimental groups is always higher than that in the control group; moreover, it increases significantly and peaks at fixed week 2, and then gradually decreases.

CONCLUSIONThe mechanical strain created by rapid maxillary expansion generates a sequence of VEGF cellular events that lead to increased vascularization and subsequent new bone formation.

Animals ; Maxilla ; Osteoblasts ; Osteogenesis ; Osteogenesis, Distraction ; Palatal Expansion Technique ; Rabbits ; Sutures ; Vascular Endothelial Growth Factor A

OBJECTIVETo analyze the effect of mandibular distraction on the maxilla growth in children with hemifacial microsomia through measurement with the posterior-anterior cephalometric X-ray films and Three-dimensional CT reconstruction images.

METHODSThe deviation angular of maxilla occlusion plane and nasal base plane from the infra-orbital plane were measured on the posterior-anterior cephalometric X-ray films in 22 patients before and half a year after operation. The vertical distance from the midpoint of 5th teeth alveolar and the lowest point of maxillary sinus to reference plane were measured on 3D reconstruction images in 15 patients. The data were statistically analyzed.

RESULTSOn posterior-anterior cephalometric X-ray films, the cant of occlusion plane were significantly reduced (P < 0.05), While the angular of nasal base plane and the infra-orbital plane had no significant change. On 3D reconstruction images, all the detection points had significantly declined except the lowest point of maxillary sinus on normal side.

CONCLUSIONSDistraction osteogenesis of mandible can promote the growth of the maxilla in children with HFM, the accelerated growth parts include alveolar bone and maxillary sinus.

Child ; Dental Occlusion ; Goldenhar Syndrome ; surgery ; Humans ; Mandible ; surgery ; Maxilla ; growth & development ; Osteogenesis, Distraction

OBJECTIVEThis study aims to analyze the biomechanical effects of four sutures that are related to maxillary growth during a maxillary protraction treatment cycle based on labiolingual appliance.

METHODSA three-dimensional simulation of labiolingual appliance was performed based on a three-dimensional finite-element maxillofacial model combined with solid-modeled sutures.

RESULTSThe displacement and stress distributions were calculated. The labiolingual appliance was suitable for patients with underbites or deep overbites. Sutura frontomaxillaris and sutura zygomaticomaxillaris were vital to the forward displacement and counterclockwise rotation of the maxillary. The changes in sutura zygomaticotemporalis and sutura palatine were not significant.

CONCLUSIONThe influences of the labiolingual appliance on the sutures during the treatment cycle of maxillary protraction can be effectively simulated by the three-dimensional finite-element maxillofacial model.

Biomechanical Phenomena ; Cranial Sutures ; Finite Element Analysis ; Humans ; Imaging, Three-Dimensional ; Malocclusion, Angle Class III ; Maxilla ; growth & development ; Orthodontic Appliances ; Skull

Maxilla and mandible have different patterns of cortical and trabecular bone and different bone mineral densities, even though both are components of the jaw bone. However, cellular differences between maxilla- and mandible derived osteoblasts (OBs) have rarely been studied. We hypothesize that maxilla- and mandible-derived OBs show different responses to 17beta-estradiol (E2), which is one of the critical factors for bone formation. This study compares skeletal site-specific cell responses between maxilla- and mandible-derived human OBs to E2.METHODS: Maxilla- and mandible-derived OBs derived from an identical donor were separately isolated from a total of five normal healthy subjects aged 18~44 years old, cultured with a treatment of 100 nM estrogen. The responses between maxilla- and mandible-derived OBs to E2 were evaluated and compared using cell proliferation, alkaline phosphatase (ALP) activity and gene expression of osteoprotegerin (OPG), ALP, insulin-like growth factor-1 (IGF-1), and estrogen receptor alpha (ERalpha).RESULTS: E2 did not have any distinct effects on the proliferation of both types of OBs. Mandible-derived OBs exhibited higher ALP activity than maxilla-derived OBs in the non-treated condition, which was common in all tested individuals. ALP activities of both types of OBs showed a minor increasing tendency with the treatment of E2, even though there was no statistical significance in some specimens. The gene expression of OB by E2 was diverse, depending on the individuals. There was increased expression of OPG, IGF-1, or ERalpha gene in the part of subjects, which was more repeated in maxilla-derived OBs. In particular, OPG or ALP induction by E appeared less frequently in mandible-derived OBs.CONCLUSION: Current results revealed that E2 affects maxilla- and mandible-derived OBs into facilitating the osteogenic process despite individual differences. Mandible-derived OBs are less sensitive to bone-forming gene expression by E2.]]>
Aged ; Alkaline Phosphatase ; Bone Density ; Cell Proliferation ; Durapatite ; Estradiol ; Estrogen Receptor alpha ; Estrogens ; Gene Expression ; Humans ; Individuality ; Insulin-Like Growth Factor I ; Jaw ; Mandible ; Maxilla ; Osteoblasts ; Osteogenesis ; Osteoprotegerin ; Tissue Donors

OBJECTIVETo analyze craniofacial growth three-dimensionally for adolescents with normal occlusion in Beijing.

METHODSOne hundred and twenty-six adolescents with normal occlusion were selected according to the criteria. The sample was divided into four age groups (53 within 4 years, 30 within 7 years, 27 within 10 years and 16 within 13 years). Information of growth was collected. Three-dimensional cephalometric system based on cone-bean CT was established.

RESULTSFrom 4 to 13 years Co-A increased (14.55 ± 1.15) mm on average on the left and (13.66 ± 1.14) mm on the right, and Co-Gn increased (22.89 ± 1.40) mm on the left and (22.82 ± 1.38) mm on the right; and U1-NA increased (2.20 ± 0.44) mm on the left and (1.60 ± 0.46) mm on the right; and CoL-CoR and GoL-GoR increased (13.31 ± 1.21) mm and (18.59 ± 1.40) mm, and N-Me increased (18.03 ± 1.32) mm.SN-PP and SN-MPL basically remained unchanged.

CONCLUSIONSAdolescents with normal occlusion in Beijing grew obviously in three-dimensions and developed harmoniously.

Adolescent ; Cephalometry ; methods ; Child ; Child, Preschool ; China ; Cone-Beam Computed Tomography ; Dental Occlusion ; Face ; anatomy & histology ; diagnostic imaging ; Facial Bones ; anatomy & histology ; diagnostic imaging ; growth & development ; Humans ; Imaging, Three-Dimensional ; Incisor ; anatomy & histology ; diagnostic imaging ; growth & development ; Mandible ; anatomy & histology ; diagnostic imaging ; growth & development ; Maxilla ; anatomy & histology ; diagnostic imaging ; growth & development ; Maxillofacial Development

OBJECTIVETo determine if locally administered bone morphogenetic protein-2 (BMP-2) and osteoprotegerin (OPG) improved osteogenesis and new bone formation by trans-sutural distraction osteogenesis.

METHODSTwenty four dogs were divided into three groups randomly and received new internal trans-sutural distraction osteogenesis treatment. Five days after operation, infusion apparatus with double-tube was inserted to submucosa near the distracted zone to deliver controlled release agent of recombinant human bone morphogenetic protein-2/poly (lactic-co-glycolic acid)/fibrin sealant (rhBMP-2/PLGA/FS) in group A and group C. Recombinant human osteoprotegerin/fibrin sealant (rhOPG/ FS) was injected three weeks later in group B and group C. Histology staining and bone histomorphometry were used to measure the changes of maxillary bone sutura after distraction for 1, 2, 4 and 6 weeks.

RESULTSNew bone formation observed in distracted zone showed a significant increase in group A and C. Transmission electron microscope showed the osteoblast and osteocyte were active with dilated rough endoplasmic reticulum and a large number of chondriosomes and Golgi complex. After distraction for 6 weeks, indexes of osteoblast of group A, B, and C were 38.5 +/- 7.7, 35.7 +/- 6.5, and 41.7 +/- 11.0, indexes of osteoclast (Ioc) were 5.9 +/- 1.0, 1.2 +/- 0.3, and 2.8 +/- 0.4, bone trabecula thicknesses were (38.36 +/- 13.28), (66.20 +/- 9.16), and (51.85 +/- 9.92) microm respectively. Increased bone density and decreased Ioc were found in group B and C.

CONCLUSIONThe new elastic distractor is effective in inducing new bone formation. BMP-2 and OPG combination acts synergistically, and leads to significant enhancement of bone formation and remodeling.

Animals ; Bone Density ; Bone Morphogenetic Protein 2 ; Dogs ; Humans ; Lactic Acid ; Maxilla ; Osteoblasts ; Osteogenesis ; Osteogenesis, Distraction ; Osteoprotegerin ; Polyesters ; Polyglycolic Acid ; Polymers ; Recombinant Proteins ; Transforming Growth Factor beta

OBJECTIVES: Dental implants installation in patients with diabetes remains controversial as altered bone healing around implants has been reported. And little is known about the biological factors involved in bone healing around implants. The present study aimed to investigate the biological markers around immediately placed implants in rats with controlled and uncontrolled diabetes. MATERIALS AND METHODS: Twenty rats (40 sites) were divided into the control, insulin-treated and diabetic groups. The rats received streptozotocin (60 mg/kg) to induce diabetes; animals in the insulin-treated group also received three units of subcutaneous slow-release insulin. Two threaded titanium alloy implant (1.2x3 mm) were placed in the extraction socket of the both maxillary first molars and allowed for healing. Bone blocks including implant were harvested at 3 days, 1, 2 and 4 weeks. The levels of bone morphogenetic protein (BMP)-4, transforming growth factor (TGF)-beta1, osteocalcin (OC) and osteonectin (ON) were measured in the peri-implant osseous samples by RT-PCR. RESULTS: The BMP-4 level increased immediately in all groups by day 3, then decreased abruptly in the control and the insulin-treated groups. However, by week 4, all groups showed mostly the same amount of BMP-4 expression. The level of TGF-beta1 also instantly increased by day 3 in the insulin-treated group. This level elevated again reaching the same values as the control group by week 4, but was not as high as the diabetic group. In addition, the expression of OC and ON in the control and insulin-treated groups was higher than that of the diabetic group at 2 weeks and 4 weeks, indicating active bone formation in these groups. CONCLUSION: The immediate placement of titanium implants in the maxilla of diabetic rat led to an unwanted bone healing response. Conclusively, the results of this study suggest that immediate implant insertion in patients with poorly controlled diabetes might be contraindicated.
Alloys ; Animals ; Biological Factors ; Biomarkers ; Bone Morphogenetic Proteins ; Dental Implants ; Humans ; Insulin ; Maxilla ; Molar ; Osteocalcin ; Osteogenesis ; Osteonectin ; Rats ; Streptozocin ; Titanium ; Transforming Growth Factor beta1 ; Transforming Growth Factors

This study is to evaluate the growth and development of the maxilla advanced by transoral distraction osteogenesis of cleft lip and palate children. Subjects are two patients diagnosed as maxillary hypoplasia with cleft lip and palate, and followed up over 5 years after distraction. At the age of 11.4 years (mean), the distraction had been rendered and periodically taken lateral cephalograms were analysed to trace the growth of the maxilla. This cephalometric study showed continuous growth and development of the distracted maxilla to be stable through long term follow-up.
Child ; Cleft Lip ; Follow-Up Studies ; Growth and Development ; Humans ; Maxilla ; Osteogenesis, Distraction ; Palate