Complex cytogenetic rearrangement in a case of unicameral bone cyst

Cytogenetic analysis of a unicameral bone cyst surgically resected in an 11-year-old boy revealed a highly complex clonal structural rearrangement involving chromosomes 4, 6, 8, 16, 21, and both 12. These findings reinforce the need for further studies on unicameral bone cysts to verify the frequency and to understand the significance of chromosome anomalies in this type of lesion.

ChronOS Inject in Children with Benign Bone Lesions – Does It Increase the Healing Rate?

Benign bone lesions in children although rare, can result in a pathological fracture. Although their etiology and pathogenesis are not yet entirely clear, the phenomenon of spontaneous healing is well known. Nevertheless, some benign bone lesions are unlikely to heal spontaneously due to the patient's age or high risk of fracture and deformity due to the lesion's location or size. The following study presents our results after treatment of these bone cysts with chronOS Inject.

Cisto ósseo traumático da mandíbula: revisão de 26 casos

O cisto ósseo traumático é uma entidade patológica caracterizada pela presença de uma cavidade óssea assintomática desprovida de revestimento epitelial, sendo raramente encontrado nos maxilares. OBJETIVO: Descrever as características clínico-cirúrgicas e radiográficas dos cistos ósseos traumáticos. MATERIAL E MÉTODO: Estudo de caráter retrospectivo dos pacientes diagnosticados com cisto ósseo traumático em um serviço de patologia oral no período de 1992 a 2007. Informações referentes às características clínicas, radiográficas e cirúrgicas foram coletadas. RESULTADOS: Vinte e seis casos de cisto ósseo traumático foram diagnosticados no período de 15 anos, 17 pertencentes ao sexo masculino e 09 ao sexo feminino. A maioria dos pacientes afetados pertencia às duas primeiras décadas de vida, não relatava sintomatologia dolorosa, bem como história de trauma na região da lesão. O padrão multilocular foi observado em apenas sete casos, dando às lesões uma aparência radiográfica tumoral. A presença de ar no interior da cavidade patológica foi relatada em aproximadamente 70% dos casos, sendo rara a presença de conteúdo serossanguíneo e seroso. CONCLUSÃO: A maior prevalência de casos em pacientes jovens, a infrequente história de trauma e o pequeno número de lesões com conteúdo serossanguíneo refletem a necessidade de se discutir a real patogênese do cisto ósseo traumático.

[The "globulomaxillary cyst" a specific entity or a myth?]

The following review investigates the term and concept of the globulomaxillary cyst as a correct clinico-pathological diagnosis to describe a so-called fissural cyst said to be caused by epithelial entrapment between the nasal and maxillary process. After analyzing the available literature it has to be concluded that neither from an embryologic nor from a clinical or pathohistological standpoint the term globulomaxillary cyst represents a real entity by itself. Therefore, globulomaxillary cysts have to be diagnosed alternatively after a thorough clinical, radiological and histological examination as other odontogenic cysts like dentigerous cysts or odontogenic keratocysts, odontogenic tumors like ameloblastoma, central giant cell tumors, solitary bone cysts, etc.

Evaluation of the maxillary sinus in panoramic radiography—a comparative study

Background: The aim of this study was to evaluate the validity and the inter- and intra-examiner reliability of panoramic-radiograph-driven findings of different maxillary sinus anatomic variations and pathologies, which had initially been prediagnosed by cone beam computed tomography (CBCT). Methods: After pairs of two-dimensional (2D) panoramic and three-dimensional (3D) CBCT images of patients having received treatment at the outpatient department had been screened, the predefinition of 54 selected maxillary sinus conditions was initially performed on CBCT images by two blinded consultants individually using a questionnaire that defined ten different clinically relevant findings. Using the identic questionnaire, these consultants performed the evaluation of the panoramic radiographs at a later time point. The results were analyzed for inter-imaging differences in the evaluation of the maxillary sinus between 2D and 3D imaging methods. Additionally, two resident groups (first year and last year of training) performed two diagnostic runs of the panoramic radiographs and results were analyzed for inter- and intra-observer reliability. Results: There is a moderate risk for false diagnosis of findings of the maxillary sinus if only panoramic radiography is used. Based on the ten predefined conditions, solely maxillary bone cysts penetrating into the sinus were frequently detected differently comparing 2D to 3D diagnostics. Additionally, on panoramic radiographs, the inter-observer comparison demonstrated that basal septa were significantly often rated differently and the intra-observer comparison showed a significant lack in reliability in detecting maxillary bone cysts penetrating into the sinus. Conclusions: Panoramic radiography provides the most information on the maxillary sinus, and it may be an adequate imaging method. However, particular findings of the maxillary sinus in panoramic imaging may be based on a rather examiner-dependent assessment. Therefore, a persistent and precise evaluation of specific conditions of the maxillary sinus may only be possible using CBCT because it provides additional information compared to panoramic radiography. This might be relevant for consecutive surgical procedures; consequently, we recommend CBCT if a precise preoperative evaluation is mandatory. However, higher radiation dose and costs of 3D imaging need to be considered. Keywords: Panoramic radiography; Cone beam computed tomography; Maxillary sinus; Inter-imaging method differences; Inter-examiner reliability; Intra-examiner reliability

Arthroscopically assisted removal of intraosseous ganglion cysts of the distal tibia

Intraosseous ganglia of the distal tibia are rare. We evaluated the feasibility of surgically treating these lesions with an arthroscopically assisted technique. Five patients with symptomatic distal tibial ganglia underwent surgical curettage and excision with this technique. All patients underwent débridement of the chondral lesion and hypertrophied synovial lining when present, probing of the portal to the ganglion, and subsequently thorough curettage with bone grafting performed through a cortical window made from a separate small incision. Biopsy confirmed the diagnosis in all patients. All patients had eventual relief of symptoms with good integration of bone graft at final followup. There were no recurrences at a minimum followup of 19 months (mean, 38.6 months; range, 19-69 months). Mean time for return to full function was 15.4 weeks (range, 8-17 weeks). There were no intraoperative or postoperative complications. The mean American Orthopaedic Foot and Ankle Society scores increased from 73 points (range, 67-77 points) preoperatively to 94 points (range, 90-100 points) postoperatively. Arthroscopically assisted surgical treatment of ganglia of the distal tibia in the appropriate patient is a reasonably simple technique that relieves symptoms and helps the patient to regain normal gait and full function with no recurrence (in our small series). LEVEL OF EVIDENCE: Level IV, case series. See Guidelines for Authors for a complete description of levels of evidence.

Does hyoid bone resection according to Sistrunk influence normal craniofacial growth? A cephalometric study

PURPOSE: To retrospectively evaluate the influence of hyoid bone resection according to Sistrunk in early age due to a thyroglossal duct cyst on craniofacial growth. MATERIALS AND METHODS: We retrospectively examined 10 patients (2 females and 8 males) having had hyoid bone resection according to Sistrunk due to thyroglossal duct cysts by lateral cephalograms taken before orthodontic treatment (mean, 17.1 years; range, 8.6-31.9 years). Surgery was carried out at a mean age of 4.4 years (range, 0.37-9.8 years). All lateral cephalograms were evaluated and traced by hand. Descriptive statistics were calculated, and data from each patient were compared individually with corresponding standard values (age and gender) from Bathia and Leighton. RESULTS: With regard to sagittal parameters, the SNB angles were by trend too small and the ANB angles were too large. However, the ratio of mandibular to maxillary length showed that the patients had a mandible that was too large or maxilla that was too small. With regard to vertical parameters, large deviations from normal values in both directions (hyperdivergent to hypodivergent pattern) could be detected when we analyzed NSL/ML', NL/ML', and NSL/NL. With regard to dental parameters, the majority of the patients had retroclined upper (IsL/NL, IsL/N-A) and lower (IiL/ML, IiL/N-B) incisors. CONCLUSIONS: Several vertical and horizontal skeletal and dental cephalometric parameters were shown to be different by trend when compared with control values. A possible negative impact on craniofacial growth potential and direction as a result of hyoid resection in early age according to Sistrunk cannot be excluded.

Aromatase deficiency in a female who is compound heterozygote for two new point mutations in the P450arom gene: impact of estrogens on hypergonadotropic hypogonadism, multicystic ovaries, and bone densitometry in childhood

We report on a female who is compound heterozygote for two new point mutations in the CYP19 gene. The allele inherited from her mother presented a base pair deletion (C) occurring at P408 (CCC, exon 9), causing a frameshift that results in a nonsense codon 111 bp (37 aa) further down in the CYP19 gene. The allele inherited from her father showed a point mutation from G-->A at the splicing point (canonical GT to mutational AT) between exon and intron 3. This mutation ignores the splice site and a stop codon 3 bp downstream occurs. Aromatase deficiency was already suspected because of the marked virilization occurring prepartum in the mother, and the diagnosis was confirmed shortly after birth. Extremely low levels of serum estrogens were found in contrast to high levels of androgens. Ultrasonographic follow-up studies revealed persistently enlarged ovaries (19.5-22 mL) during early childhood (2 to 4 yr) which contained numerous large cysts up to 4.8 x 3.7 cm and normal-appearing large tertiary follicles already at the age of 2 yr. In addition, both basal and GnRH-induced FSH levels remained consistently strikingly elevated. Low-dose estradiol (E2) (0.4 mg/day) given for 50 days at the age of 3 6/12 yr resulted in normalization of serum gonadotropin levels, regression of ovarian size, and increase of whole body and lumbar spine (L1-L4) bone mineral density. The FSH concentration and ovarian size returned to pretreatment levels shortly (150 days) after cessation of E2 therapy. Therefore, we recommend that affected females be treated with low-dose E2 in amounts sufficient to result in physiological prepubertal E2 concentrations using an ultrasensitive estrogen assay. However, E2 replacement needs to be adjusted throughout childhood and puberty to ensure normal skeletal maturation and adequate adolescent growth spurt, normal accretion of bone mineral density, and, at the appropriate age, female secondary sex maturation.

Fresh-frozen vs irradiated allograft bone in orthopaedic reconstructive surgery

The use of allograft bone is increasingly common in orthopaedic reconstruction procedures. The optimal method of preparation of allograft bone is subject of great debate. Proponents of fresh-frozen graft cite improved biological and biomechanical characteristics relative to irradiated material, whereas fear of bacterial or viral transmission warrants some to favour irradiated graft. Careful review of the literature is necessary to appreciate the influence of processing techniques on bone quality. Whereas limited clinical trials are available to govern the selection of appropriate bone graft, this review presents the argument favouring the use of fresh-frozen bone allograft as compared to irradiated bone.

Lateral bone density variations in the scoliotic spine

Adolescent Idiopathic Scoliosis (AIS) is the most common deformity of the spine, affecting 2-4% of the population. Previous studies have shown that the vertebrae in scoliotic spines undergo abnormal shape changes, however there has been little exploration of how AIS affects bone density distribution within the vertebrae. Existing pre-operative CT scans of 53 female idiopathic scoliosis patients with right-sided main thoracic curves were used to measure the lateral (right to left) bone density profile at mid-height through each vertebral body. This study demonstrated that AIS patients have a marked convex/concave asymmetry in bone density for vertebral levels at or near the apex of the scoliotic curve. To the best of our knowledge, the only previous studies of bone density distribution in AIS are those of Périé et al [1,2], who reported a coronal plane ‘mechanical migration’ of 0.54mm toward the concavity of the scoliotic curve in the lumbar apical vertebrae of 11 scoliosis patients. This is comparable to the value of 0.8mm (4%) in our study, especially since our patients had more severe scoliotic curves. From a bone adaptation perspective, these results suggest that the axial loading on the scoliotic spine is strongly asymmetric.

Effect of bone graft type on fusion rates following endoscopic anterior scoliosis correction

Bone graft is generally considered fundamental in achieving solid fusion in scoliosis correction and pseudarthrosis following instrumentation may predispose to implant failure. In endoscopic anterior-instrumented scoliosis surgery, autologous rib or iliac crest graft has been utilised traditionally but both techniques increase operative duration and cause donor site morbidity. Allograft bone and bone- morphogenetic-protein alternatives may improve fusion rates but this remains controversial. This study's objective was to compare two-year postoperative fusion rates in a series of patients who underwent endoscopic anterior instrumentation for thoracic scoliosis utilising various bone graft types. Significantly better rates of fusion occurred in endoscopic anterior instrumented scoliosis correction using femoral allograft compared to autologous rib-heads and iliac crest graft. This may be partly explained by the difficulty obtaining sufficient quantities of autologous graft. Lower fusion rates in the autologous graft group appeared to predispose to rod fracture although the clinical consequence of implant failure is uncertain.

Implication of 3D culture system for study of prostate cancer (CaP) mediated bone metastasis

Introduction : For the past decade, three dimensional (3D) culture has served as a foundation for regenerative medicine study. With an increasing awareness of the importance of cell-cell and cell-extracellular matrix interactions which are lacking in 2D culture system, 3D culture system has been employed for many other applications namely cancer research. Through development of various biomaterials and utilization of tissue engineering technology, many in vivo physiological responses are now better understood. The cellular and molecular communication of cancer cells and their microenvironment, for instance can be studied in vitro in 3D culture system without relying on animal models alone. Predilection of prostate cancer (CaP) to bone remains obscure due to the complexity of the mechanisms and lack of proper model for the studies. In this study, we aim to investigate the interaction between CaP cells and osteoblasts simulating the natural bone metastasis. We also further investigate the invasiveness of CaP cells and response of androgen sensitve CaP cells, LNCaP to synthetic androgen.----- Method : Human osteoblast (hOB) scaffolds were prepared by seeding hOB on medical grade polycaprolactone-tricalcium phosphate (mPLC-TCP) scaffolds and induced to produce bone matrix. CaP cell lines namely wild type PC3 (PC3-N), overexpressed prostate specific antigen PC3 (PC3k3s5) and LNCaP were seeded on hOB scaffolds as co-cultures. Morphology of cells was examined by Phalloidin-DAPI and SEM imaging. Gelatin zymography was performed on the 48 hours conditioned media (CM) from co-cultures to determine matrix metalloproteinase (MMP) activity. Gene expression of hOB/LNCaP co-cultures which were treated for 48 hours with 1nM synthetic androgen R1881 were analysed by quantitative real time PCR (qRT-PCR).----- Results : Co-culture of PCC/hOB revealed that the morphology of PCCs on the tissue engineered bone matrix varied from homogenous to heterogenous clusters. Enzymatically inactive pro-MMP2 was detected in CM from hOBs and PCCs cultured on scaffolds. Elevation in MMP9 activity was found only in hOB/PC3N co-culture. hOB/LNCaP co-culture showed increase in expression of key enzymes associated with steroid production which also corresponded to an increase in prostate specific antigen (PSA) and MMP9.----- Conclusions : Upregulation of MMP9 indicates involvement of ECM degradation during cancer invasion and bone metastases. Expression of enzymes involved in CaP progression, PSA, which is not expressed in osteoblasts, demonstrates that crosstalk between PCCs and osteoblasts may play a part in the aggressiveness of CaP. The presence of steroidogenic enzymes, particularly, RDH5, in osteoblasts and stimulated expression in co-culture, may indicate osteoblast production of potent androgens, fuelling cancer cell proliferation. Based on these results, this practical 3D culture system may provide greater understanding into CaP mediated bone metastasis. This allows the role of the CaP/hOB interaction with regards to invasive property and steroidogenesis to be further explored.