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This study aimed to test age-related changes in sternal fusion and sternal-rib cartilage ossification on multi-slice computed tomography (MSCT) images of the Croatian population. The additional aim was to develop models to estimate age and provide an interface for the model's application and validation. This retrospective study was conducted on 144 MSCT images of the sternal region, and the developed models were tested on 36 MSCT images. We scored manubrium-mesosternal joint fusion (FM), xiphoid process and mesosternum fusion (FX), ossification of the first costal cartilage (OF), ossification of the second to seventh costal cartilages at the rib ends (OR), and ossification of the second to seventh costal cartilages at the sternal ends (OS). All sternal-rib cartilage ossification phases and sternal body and xiphoid process fusion scores showed statistically significant age differences (P < 0.001), except manubrium-mesosternal joint fusion. The final model that combined regression and classification using FM, FX, OR, OS, and sex obtained a 95% prediction interval (PI) coverage of 94.46% on the cross-validation (cv) and 91.67% on the test set with an average PI width of 42.29 and 42.95 years respectively. We also developed a Python Flask app called CroSterna: Age estimation from sternal fusion and rib ossification in the Croatian population ( https://crosterna.onrender.com/ ) to facilitate the estimation for professionals., Competing Interests: Declarations. Ethical approval: This study was approved by the ethical committee of the University Hospital of Split on October 26th, 2023 (Class: 500 − 13/23 − 01/205; Number: 2181-147-01-06/LJ.Z.-23-02), in accordance with the Declaration of Helsinki. Consent to participate: Not applicable. Clinical trial number: Not applicable. Declaration of generative AI and AI-assisted technologies in the writing process: While preparing this work, the authors used ChatGPT Data Analysis for guidance during the construction of models and code development. After using this tool, the authors reviewed and edited the content as needed and took full responsibility for the content of the published article. Competing interests: The authors have no competing interests to declare that are relevant to the content of this article., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

There is considerable variation in the gross morphology and tissue properties among the bones of human infants, children, adolescents, and adults. Using 18 known-age individuals (n female  = 8, n male  = 9, n unknown  = 1; birth to 21 years old), from a well-documented cemetery collection, Spitalfields Christ Church, London, UK, this study explores growth-related changes in cortical and trabecular bone microstructure. Micro-CT scans of mid-shaft middle thoracic ribs are used for quantitative analysis. Results are then compared to previously quantified conventional histomorphometry of the same sample. Total area (Tt.Ar), cortical area (Ct.Ar), cortical thickness (Ct.Th), and the major (Maj.Dm) and minor (Min.Dm) diameters of the rib demonstrate positive correlations with age. Pore density (Po.Dn) increases, but age-related changes to cortical porosity (Ct.Po) appear to be non-linear. Trabecular thickness (Tb.th) and trabecular separation (Tb.Sp) increase with age, whereas trabecular bone pattern factor (Tb.Pf), structural model index (SMI), and connectivity density (Conn.D) decrease with age. Sex-based differences were not identified for any of the variables included in this study. Some samples display clear evidence of diagenetic alteration without corresponding changes in radiopacity, which compromises the reliability of bone mineral density (BMD) data in the study of past populations. Cortical porosity data are not correlated with two-dimensional measures of osteon population density (OPD). This suggests that unfilled resorption spaces contribute more significantly to cortical porosity than do the Haversian canals of secondary osteons. Continued research using complementary imaging techniques and a wide array of histological variables will increase our understanding of age- and sex-specific ontogenetic patterns within and among human populations., (© 2019 Anatomical Society.)

Thallium (Tl) is a highly toxic metal for human beings; higher amounts found in diverse fluids of pregnant women are associated with low birth weight and preterm birth. However, experimental data concerning their effects on the embryonic development of mammalian organisms are limited. Hence, in the present work, TI(I) acetate of 0, 4.6, 9.2, or 18.5 mg/kg body weight were administered by intraperitoneal injection to groups of 10 pregnant CD-1 mice on the 7th gestational day, and animals were sacrificed on day 18 of gestation. The fetuses obtained showed some variations, such as trunk bent over (18.5 mg/kg), tail variations (all doses), forelimbs malrotation and hind limbs (all doses). Skeletal examination of the fetuses showed a delay in the ossification of skull bones, ribs, and limbs (all doses). In conclusion, the Intraperitoneal injection of Tl(I) acetate to pregnant mice induced morphological variations and a delay of the fetus ossification., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Thoracolumbar supernumerary ribs (TSRs) are classified as less severe skeletal anomalies in rat developmental toxicity studies, although their incidence is relatively high in rodent studies. To investigate the characteristics of the critical window for chemically-induced TSR, in this study, rats were administered 5-fluorocytocine (5-FC) or sodium salicylate (SAL) at one of three time periods on gestational day (GD) 9, early morning (7:00 am), midday (12:00 pm to 1:00 pm), or late afternoon (4:00 pm or 7:00 pm). The incidence of TSR and other anomalies were assessed in GD20 fetuses. A single treatment with both chemicals on GD9-induced TSR, with the incidence highest when administered at 7:00 Am, decreasing gradually when administered later. This trajectory was clearer in rats treated with 5-FC than with SAL. The critical period of TSR induction is shorter in rats administered 5-FC than SAL. The characteristics of the critical window may cause variability in the incidence of TSR observed in developmental toxicity studies., (© 2018 Japanese Teratology Society.)

Tendons are an essential part of the musculoskeletal system, connecting muscle and skeletal elements to enable force generation. The transcription factor scleraxis marks vertebrate tendons from early specification. Scleraxis -null mice are viable and have a range of tendon and bone defects in the trunk and limbs but no described cranial phenotype. We report the expression of zebrafish scleraxis orthologs: scleraxis homolog ( scx)-a and scxb in cranial and intramuscular tendons and in other skeletal elements. Single mutants for either scxa or scxb, generated by clustered regularly interspaced short palindromic repeats (CRISPR)/CRISPR-associated protein 9 (Cas9), are viable and fertile as adult fish. Although scxb mutants show no obvious phenotype, scxa mutant embryos have defects in cranial tendon maturation and muscle misalignment. Mutation of both scleraxis genes results in more severe defects in cranial tendon differentiation, muscle and cartilage dysmorphogenesis and paralysis, and lethality by 2-5 wk, which indicates an essential function of scleraxis for craniofacial development. At juvenile and adult stages, ribs in scxa mutants fail to mineralize and/or are small and heavily fractured. Scxa mutants also have smaller muscle volume, abnormal swim movement, and defects in bone growth and composition. Scleraxis function is therefore essential for normal craniofacial form and function and vital for fish development.-Kague, E., Hughes, S. M., Lawrence, E. A., Cross, S., Martin-Silverstone, E., Hammond, C. L., Hinits, Y. Scleraxis genes are required for normal musculoskeletal development and for rib growth and mineralization in zebrafish.

Most bones in mammals display a limited capacity for natural large-scale repair. The ribs are a notable exception, yet the source of their remarkable regenerative ability remains unknown. Here, we identify a Sox9 -expressing periosteal subpopulation that orchestrates large-scale regeneration of murine rib bones. Deletion of the obligate Hedgehog co-receptor, Smoothened , in Sox9 -expressing cells prior to injury results in a near-complete loss of callus formation and rib bone regeneration. In contrast to its role in development, Hedgehog signaling is dispensable for the proliferative expansion of callus cells in response to injury. Instead, Sox9-positive lineage cells require Hh signaling to stimulate neighboring cells to differentiate via an unknown signal into a skeletal cell type with dual chondrocyte/osteoblast properties. This type of callus cell may be critical for bridging large bone injuries. Thus despite contributing to only a subset of callus cells, Sox9 -positive progenitors play a major role in orchestrating large-scale bone regeneration., Editorial Note: This article has been through an editorial process in which the authors decide how to respond to the issues raised during peer review. The Reviewing Editor's assessment is that all the issues have been addressed (see decision letter)., Competing Interests: SK, MS, VV, NT, NH, JL, JC, FM No competing interests declared, (© 2019, Kuwahara et al.)

This study proposes the description of the development of the postcranial axial skeleton, including vertebrae, gastralium, ribs, sternum, and interclavicle, in Melanosuchus niger. Six nests were marked and two eggs removed from each nest at 24-hr intervals until hatching. For posthatching evaluation, 30 hatchlings were kept in captivity and one exemplar was euthanized at three-day intervals. Samples were diaphanized using potassium hydroxide (KOH), alizarin red S, and Alcian blue. A routinely generally used method was applied for histological evaluation. It was difficult to define in which vertebrae the development of cartilaginous centers began, but it was possible to observe that this condensation advanced in the craniocaudal direction. The condensation started in the vertebral arches and was visibly stronger in the cervical and dorsal regions, advancing to the lumbar, sacral and, last, to the caudal region. The atlas showed a highly different morphology compared with the other cervical vertebrae, with a short intercenter, two neural arches, and a proatlas. The ossification process began in the body of cervical vertebrae III to VIII and alizarin retention decreased in the last vertebrae, indicating a craniocaudal direction in bone development, similar to cartilage formation. In the histological sections of gastralium and interclavicles of M. niger at several development stages, it was possible to observe that these elements showed intramembranous development. Anat Rec, 301:607-623, 2018. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Costal cartilage ossification on chest plate radiographs is one of the useful methods in age estimation for adults. This study was performed in 136 remains yielding a regression formula for age estimation in Thai male population. Eight features on chest plate imaging were scored based on Garvin's method. Composite scores were calculated by summation of all scores and analyzed to generate the regression of age as: Age=16.664×e 0.161 (composite score) with a 95% prediction interval. The predicted age intervals in all composite scores were overlapping except for score 0 and 7. It could be concluded that if all features are absent/present, the person is likely to be less/more than 29years old., (Copyright © 2017. Published by Elsevier B.V.)

Background: Intermuscular bones (IBs) and ribs both are a part of skeletal system in teleosts, but with different developing process. The chemical composition of fish IBs and ribs as well as the underlying mechanism about their development have not been investigated. In the present study, histological structures showed that one bone cavity containing osteoclasts were existed in ribs, but not in IBs of Megalobrama amblycephala. We constructed the first proteomics map for fish bones including IBs and ribs, and identified the differentially expressed proteins between IBs and ribs through iTRAQ LC-MS/MS proteomic analysis., Results: The proteins extracted from IBs and ribs at 1- to 2-year old M. amblycephala were quantified 2,342 proteins, with 1,451 proteins annotated with GO annotation in biological processes, molecular function and cellular component. A number of bone related proteins as well as pathways were identified in the study. A total of 93 and 154 differently expressed proteins were identified in comparison groups of 1-IB-vs-1-Rib and 2-IB-vs-2-Rib, which indicated the obvious differences of chemical composition between these two bone tissues. The two proteins (vitronectin b precursor and matrix metalloproteinase-2) related to osteoclasts differentiation were significantly up-regulated in ribs compared with IBs (P < 0.05), which was in accordance with the results from histological structures. In comparison groups of 1-IB-vs-2-IB and 1-Rib-vs-2-Rib, 33 and 51 differently expressed proteins were identified and the function annotation results showed that these proteins were involved in regulating bone development and differentiation. Subsequently, 11 and 13 candidate proteins in comparison group of 1-IB-vs-1-Rib and 1-IB-vs-2-IB related to bone development were validated by MRM assays., Conclusions: Our present study suggested the different key proteins involved in the composition of fish ribs and IBs as well as their growth development. These findings could provide important clues towards further understanding of fish skeletal system and the roles of proteins playing in regulating diverse biological processes in fish.

Cerebro-Costo-Mandibular syndrome (CCMS) is a rare autosomal dominant condition comprising branchial arch-derivative malformations with striking rib-gaps. Affected patients often have respiratory difficulties, associated with upper airway obstruction, reduced thoracic capacity, and scoliosis. We describe a series of 12 sporadic and 4 familial patients including 13 infants/children and 3 adults. Severe micrognathia and reduced numbers of ribs with gaps are consistent findings. Cleft palate, feeding difficulties, respiratory distress, tracheostomy requirement, and scoliosis are common. Additional malformations such as horseshoe kidney, hypospadias, and septal heart defect may occur. Microcephaly and significant developmental delay are present in a small minority of patients. Key radiological findings are of a narrow thorax, multiple posterior rib gaps and abnormal costo-transverse articulation. A novel finding in 2 patients is bilateral accessory ossicles arising from the hyoid bone. Recently, specific mutations in SNRPB, which encodes components of the major spliceosome, have been found to cause CCMS. These mutations cluster in an alternatively spliced regulatory exon and result in altered SNRPB expression. DNA was available from 14 patients and SNRPB mutations were identified in 12 (4 previously reported). Eleven had recurrent mutations previously described in patients with CCMS and one had a novel mutation in the alternative exon. These results confirm the specificity of SNRPB mutations in CCMS and provide further evidence for the role of spliceosomal proteins in craniofacial and thoracic development., (© 2016 Wiley Periodicals, Inc.)

Background/purpose: We compared the costal cartilage and rib length between prepubertal patients with symmetric pectus excavatum and age- and sex-matched controls without anterior chest wall depression to evaluate if rib overgrowth is a contributing factor for pectus excavatum, Methods: The sample included 18 prepubertal patients <10years old with symmetric pectus excavatum and 18 age-and sex-matched controls without chest wall deformity. The full lengths of the fourth to sixth ribs and costal cartilage were measured using three-dimensional volume-rendered computed tomography and curved multiplanar reformatting techniques. The rib and costal cartilage lengths, total combined rib and costal cartilage length, and costal index ([length of cartilage/length of rib]×100 [%]) at the fourth to sixth levels were compared between the groups., Results: The rib lengths in the patient group were significantly longer than in the control group for the 6th right rib and 4th, 5th, and 6th left ribs. The costal cartilage lengths and costal indices were not different between two groups., Conclusions: In patients with symmetric pectus excavatum aged <10years old, several of the ribs were longer than those of controls, suggesting that abnormal rib overgrowth may be a contributing factor responsible for pectus excavatum rather than cartilage overgrowth., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: The optimal age at which to initiate for auricular reconstruction is controversial. Rib cartilage growth is closely related to age and determines the feasibility and outcomes of auricular reconstruction. We developed a method to guide the timing of auricular reconstruction in children with microtia ranging in age from 5 to 10 years., Methods: Rib cartilage and the healthy ear were assessed using low-dose multi-slice computed tomography. The lengths of the eighth rib cartilage and the helix of the healthy ear (from the helical crus to the joint of the helix and the earlobe) were measured. Surgery was performed when the two lengths were approximately equal., Results: The preoperative eighth rib measurements significantly correlated with the intraoperative measurements (P < 0.05). From 5 to 10 years of age, eighth rib growth was not linear. In 76 (62.8%) of 121 patients, the eighth rib length was approximately equal to the helix length in the healthy ear; satisfactory outcomes were achieved in these patients. In 18 (14.9%) patients, the eighth rib was slightly shorter than the helix, helix fabrication was accomplished by adjusting the length of the helical crus of stent, and satisfactory outcomes were also achieved. Acceptable outcomes were achieved in 17 (14.0%) patients in whom helix fabrication was accomplished by cartilage splicing. In 9 (7.4%) patients with insufficient rib cartilage length, the operation was delayed. In one (0.8%) patient with insufficient rib cartilage length, which left no cartilage for helix splicing, the result was unsatisfactory., Conclusions: Eighth rib cartilage growth is variable. Rib cartilage assessment relative to the healthy ear can guide auricular reconstruction and personalize treatment in young patients with microtia.

Background: We conducted a cross-sectional study to quantify the developmental changes of the thoracic cage in patients with pectus excavatum (PE)., Methods: The preoperative posteroanterior standing chest radiographs (chest PAs) of 1,197 consecutive patients with PE, together with 1,661 age- and sex-matched minor surgery patients, as a control group, who were seen between June 2005 and February 2013, were reviewed. The maximum width of each rib pair and chest height (H) were measured on the chest PA., Results: In the PE group, the normal thoracic contour in younger patients was replaced by a characteristic can-shaped chest wall, which showed protrusion of the upper ribs, an increased H, and a straightened lateral border of the chest cage, as they grew into adulthood. The chest height difference between the PE and control groups increased progressively, from the age of 8 years and most significantly from age 13 to 17 years. No difference was observed in the middle and lower rib widths. Sex did not influence these trends., Conclusion: The PE chest wall shows a significant increment in chest height, and upper rib width starts during the period of rapid growth and is maintained into adulthood. The thoracic deformity in patients with PE includes more than just the sternal deformity., (Georg Thieme Verlag KG Stuttgart · New York.)

Background: To evaluate whether the overgrowth of costal cartilage exists in patients with pectus excavatum, we compared the length of the costal cartilage and ribs between patients with asymmetric pectus excavatum and controls without chest wall deformity using three-dimensional computed tomography., Materials and Methods: Nineteen adult patients with asymmetric pectus excavatum and 19 age and sex matched controls without chest wall deformity were enrolled. We measured the full lengths of the fourth to sixth ribs and costal cartilage using three-dimensional volume-rendered computed tomography images and curved multiplanar reformatting techniques. The lengths of ribs and costal cartilage, their summations, and the costal index ([length of cartilage/length of rib] × 100 [%]) were compared on the asymmetrically depressed side of patients (Group A), the opposite side of the same patients (Group B), and controls (Group C) at the fourth to sixth levels., Results: The lengths of the ribs of groups A and B were significantly longer (p < 0.001) than those of group C (299.4 ± 14.9 mm vs. 302.9 ± 15.3 mm vs. 288.9 ± 12.2 at the fourth level, 312.3 ± 14.1 mm vs. 318.4 ± 14.6 mm vs. 303.2 ± 12.7 mm at the fifth level, and 322.2 ± 17.2 mm vs. 325.2 ± 17.5 mm vs. 309.4 ± 12.3 mm at the sixth level). The costal cartilage lengths did not differ (p > 0.05) among the three groups (53.1 ± 7.3 mm vs. 54.6 ± 8.6 mm vs. 52.9 ± 5.2 at the fourth level, 71.9 ± 9.6 mm vs. 72.3 ± 9.9 mm vs. 69.2 ± 7.1 mm at the fifth level, and 100.1 ± 15.2 mm vs. 104.2 ± 15.8 mm vs. 99.1 ± 9.1 mm at sixth level). The summations of the rib and costal cartilage lengths were longer in groups A and B than in group C. The costal indices were not different among the three groups at the fourth, fifth, and sixth rib levels., Conclusion: In patients who had asymmetric pectus excavatum with a ≥ 21-degree angle of sternal rotations, the ribs but not the costal cartilage were longer than those of controls. These findings suggest that cartilage overgrowth is not the main factor responsible for asymmetric pectus excavatum, and it could instead be related to abnormal rib growth., (Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: We compared the length of costal cartilage and rib between patients with symmetric pectus carinatum and controls without anterior chest wall protrusion, using a 3-dimensional (3D) computed tomography (CT) to evaluate whether the overgrowth of costal cartilage exists in patients with pectus carinatum., Subjects and Methods: Twenty-six patients with symmetric pectus carinatum and matched twenty-six controls without chest wall protrusion were enrolled. We measured the full lengths of the 4th-6th ribs and costal cartilages using 3-D volume rendering CT images and the curved multiplanar reformatted (MPR) techniques. The lengths of ribs and costal cartilages, the summation of rib and costal cartilage lengths, and the costal index [length of cartilage/length of rib * 100 (%)] were compared between the patients group and the control group at 4th-6th levels., Results: The lengths of costal cartilage in patient group were significantly longer than those of control group at 4th, 5th and 6th rib level. The lengths of ribs in patient group were significantly shorter than those of control group at 4th, 5th and 6th rib level. The summations of rib and costal cartilage lengths were not longer in patients group than in control group. The costal indices were significantly larger in patients group than in control groups at 4th, 5th and 6th rib level., Conclusion: In patients with symmetric pectus carinatum, the lengths of costal cartilage were longer but the lengths of rib were shorter than those of controls. These findings may supports that the overgrowth of costal cartilage was not the only factor responsible for pectus carinatum., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Recurrent symptoms of thoracic outlet syndrome (TOS) after first rib resection have varying etiologies. Regrowth of a first rib is a rare event. Recurrent symptoms in the presence of a regrown rib strongly suggest a causal relationship. We report our experience with recurrent symptoms of TOS and regrown first ribs., Methods: We identified patients with recurrent TOS symptoms and regrown first ribs presented between 1995 and 2012. Details regarding their presentation, evaluation, and treatment were gathered., Results: Eight patients (6 women and 2 men) presenting with recurrent TOS symptoms and regrown first ribs underwent 10 decompression surgeries. Prior surgeries included supraclavicular first rib resection (5), transaxillary first rib resection (5), scalenectomy (5), cervical rib resection (1). The average period between initial surgery and reoperation was 4.7 years. Average age at current presentation was 40.8 years (range 29-52). All patients (8) represented with neurogenic symptoms and 1 patient with concomitant venous TOS symptoms. Presenting symptoms included pain (8), numbness and tingling (7), weakness (6), headache (2), and venous congestion (3). Initial treatment included physical therapy in all. Preoperative assessment included chest X-rays (8), magnetic resonance imaging (7), electrodiagnostic studies (8), venography (2), and anterior scalene muscle block (2). Surgical approach included transaxillary resection of the regrown first rib (10), neurolysis of brachial plexus (10), scalenectomy (5), and lysis of subclavian vein (1). After an average follow-up of 10.8 months, resolution of symptoms included 4 complete and 4 partial., Conclusions: Regrowth of the first rib is a rare event. There is a concordance between a regrown rib and TOS symptoms. Patients presenting with recurrent TOS symptoms and a regrown first rib have a high probability of improvement with resection of the regrown rib., (Copyright © 2014 Elsevier Inc. All rights reserved.)

We present the case of an 8-year-old girl who suffered from acute myeloid leukaemia-related necrotising fasciitis in her early childhood resulting in severe skin and soft-tissue loss with subsequent circumferential thoracic scar formation. While tissue expander use yielded successful skin replacement for median scar areas, ribs were covered with ultrathin skin envelope at the lateral thoracic wall leading to growth and shoulder abduction impairment. Skin and soft-tissue reconstruction of these extended rib areas was aimed for in a one-step approach. Both lateral thoracic walls were successfully reconstructed with free microsurgical transfer of large pre-expanded tensor fasciae latae flaps, in order to enable future thoracic growth., (Copyright © 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Objective: Careful operative timing is required for children undergoing microtia repair using autologous costochondral grafting. This operation is performed as early as age 6 in efforts to treat children before school matriculation while allowing for sufficient rib growth. There remains a paucity of data regarding cartilaginous growth of the ribs and synchondrosis routinely harvested during microtia repair. This study employs CT imaging to generate normative costochondral growth characteristics in children., Study Design: A population-based study was performed., Setting: Tertiary care children's hospital., Subjects and Methods: Chest CTs were reviewed in 360 children ages 3 to 20 years. Measurements included: length of ribs 6, 7, and 8 and the height and width of the synchondrosis between ribs 6 and 7. Growth charts are presented for gender and laterality., Results: At age 6: ribs 6, 7, and 8 measure 5.96 ± 0.69, 7.79 ± 0.84, and 6.33 ± 1.01 cm, respectively. In adulthood the mean length of ribs 6, 7, and 8 are 8.29 ± 1.00, 11.10 ± 1.19, and 8.95 ± 1.99 cm, respectively. The vertical height of the synchondrosis at years 6 and 20 are 2.42 ± 0.39 and 3.59 ± 0.53 cm, respectively. Ribs 6, 7, and 8 as well as the synchondrosis grow in a nearly linear fashion., Conclusions: Cartilaginous growth of ribs 6 to 8 during early childhood is nearly linear. Synchondrosis height approaches adult auricle width at 8 years. Rib size is consistently larger in males and on the left side. These data are useful for the pediatric otolaryngologist and facial plastics and reconstructive surgeon performing microtia surgery.

Study Design: Retrospective case series., Objective: To report the outcomes of distraction-based, growth-sparing spinal instrumentation in patients with skeletal dysplasia., Summary of Background Data: Patients with skeletal dysplasia with spinal deformity often undergo early fusion, further compromising an already small chest. Nonfusion techniques may provide a safe alternative and allow for thoracic growth., Methods: Between 2004 and 2010, 12 children with a diagnosis of various types of skeletal dysplasia underwent growth-sparing spinal instrumentation for severe spinal deformities. The mean duration of treatment with growing rods was 57 months (42-84 mo). Nine patients were treated with growing rods (8 dual, 1 single), and 3 were treated with vertical expandable prosthetic titanium rib (VEPTR; Synthes). Preoperative, initial postoperative, and final follow-up anteroposterior and lateral spine radiographs were measured for magnitude of deformity, junctional kyphosis, and implant failure., Results: The major curve Cobb angle improved from a mean of 79° preoperatively to a mean of 41° at the last follow-up (52%). There was a decrease in mean thoracic kyphosis from 77° preoperatively to 64° at final follow-up and an increase in mean lumbar lordosis from 58° preoperatively to 63° at final follow-up. The mean space available for the lungs increased by 26 mm on the concave and 24 mm on the convex side. Six patients required revision surgery for proximal junctional kyphosis. There were 4 rod failures and 6 hook and 8 screw dislodgements. One patient with vertical expandable prosthetic titanium rib had failed rib fixation that required revision., Conclusion: Growth-sparing spinal instrumentation in patients with skeletal dysplasia and severe spinal deformity has a high complication and revision rate, and surgeons should closely monitor these patients. The complication rate is comparable with previous reports on patients with other diagnoses. However, deformities were well controlled, some trunk growth was achieved, and fusion surgery was delayed in all cases., Level of Evidence: 4.

The difficulties in quantifying the 3D form and spatial relationships of the skeletal components of the ribcage present a barrier to studies of the growth of the thoracic skeleton. Thus, most studies to date have relied on traditional measurements such as distances and indices from single or few ribs. It is currently known that adult-like thoracic shape is achieved early, by the end of the second postnatal year, with the circular cross-section of the newborn thorax transforming into the ovoid shape of adults; and that the ribs become inclined such that their anterior borders come to lie inferior to their posterior. Here we present a study that revisits growth changes using geometric morphometrics applied to extensive landmark data taken from the ribcage. We digitized 402 (semi) landmarks on 3D reconstructions to assess growth changes in 27 computed tomography-scanned modern humans representing newborns to adults of both sexes. Our analyses show a curved ontogenetic trajectory, resulting from different ontogenetic growth allometries of upper and lower thoracic units. Adult thoracic morphology is achieved later than predicted, by diverse modifications in different anatomical regions during different ontogenetic stages. Besides a marked increase in antero-posterior dimensions, there is an increase in medio-lateral dimensions of the upper thorax, relative to the lower thorax. This transforms the pyramidal infant thorax into the barrel-shaped one of adults. Rib descent is produced by complex changes in 3D curvature. Developmental differences between upper and lower thoracic regions relate to differential timings and rates of maturation of the respiratory and digestive systems, the spine and the locomotor system. Our findings are relevant to understanding how changes in the relative rates of growth of these systems and structures impacted on the development and evolution of modern human body shape.

Better understanding of the effects of growth on children's bones and cartilage is necessary for clinical and biomechanical purposes. The aim of this study is to define the 3D geometry of children's rib cages: including sternum, ribs and costal cartilage. Three-dimensional reconstructions of 960 ribs, 518 costal cartilages and 113 sternebrae were performed on thoracic CT scans of 48 children, aged 4 months to 15 years. The geometry of the sternum was detailed and nine parameters were used to describe the ribs and rib cages. A "costal index" was defined as the ratio between cartilage length and whole rib length to evaluate the cartilage ratio for each rib level. For all children, the costal index decreased from rib level 1 to 3 and increased from level 3 to 7. For all levels, the cartilage accounted for 45-60 % of the rib length, and was longer for the first years of life. The mean costal index decreased by 21 % for subjects over 3-year old compared to those under three (p < 10(-4)). The volume of the sternebrae was found to be highly age dependent. Such data could be useful to define the standard geometry of the pediatric thorax and help to detect clinical abnormalities.

Primary cilia are present on most cell types including chondrocytes. Dysfunction of primary cilia results in pleiotropic symptoms including skeletal dysplasia. Previously, we showed that deletion of Ift88 and subsequent depletion of primary cilia from chondrocytes resulted in disorganized columnar structure and early loss of growth plate. To understand underlying mechanisms whereby Ift88 regulates growth plate function, we compared gene expression profiles in normal and Ift88 deleted growth plates. Pathway analysis indicated that Hedgehog (Hh) signaling was the most affected pathway in mutant growth plate. Expression of the Wnt antagonist, Sfrp5, was also down-regulated. In addition, Sfrp5 was up-regulated by Shh in rib chondrocytes and regulation of Sfrp5 by Shh was attenuated in mutant cells. This result suggests Sfrp5 is a downstream target of Hh and that Ift88 regulates its expression. Sfrp5 is an extracellular antagonist of Wnt signaling. We observed an increase in Wnt/β-catenin signaling specifically in flat columnar cells of the growth plate in Ift88 mutant mice as measured by increased expression of Axin2 and Lef1 as well as increased nuclear localization of β-catenin. We propose that Ift88 and primary cilia regulate expression of Sfrp5 and Wnt signaling pathways in growth plate via regulation of Ihh signaling., (Copyright © 2012 Orthopaedic Research Society.)

129/SvEv mice with a loss-of-function mutation in the heterotrimeric G protein α-subunit gene Gnai3 have fusions of ribs and lumbar vertebrae, indicating a requirement for Gα(i) (the "inhibitory" class of α-subunits) in somite derivatives. Mice with mutations of Gnai1 or Gnai2 have neither defect, but loss of both Gnai3 and one of the other two genes increases the number and severity of rib fusions without affecting the lumbar fusions. No myotome defects are observed in Gnai3/Gnai1 double-mutant embryos, and crosses with a conditional allele of Gnai2 indicate that Gα(i) is specifically required in cartilage precursors. Penetrance and expressivity of the rib fusion phenotype is altered in mice with a mixed C57BL/6 × 129/SvEv genetic background. These phenotypes reveal a previously unknown role for G protein-coupled signaling pathways in development of the axial skeleton.

Effects of etidronate on the calcification of scales and ribs were investigated in goldfish. Daily intraperitoneal injections of etidronate at doses of 1 and 10 mgP/kg body weight for 2 weeks inhibited calcification of ontogenic scales and ribs without affecting the accretion of organic matrices. Removal of some scales induced their regeneration within the two-week period. Their newly formed organic matrix was fully uncalcified in fish treated with 10 mgP/kg, whereas in those treated with 1 mgP/kg, the regenerating scales were only partially calcified. Daily administration of etidronate 10 mgP/kg resulted in an increase of body weight. These results suggested that the inhibitory effect of etidronate on the calcification of osseous tissues in mammals can be expected also on comparable tissues in fishes. An appropriate dose of etidronate that inhibits hard tissue calcification but not affects the body growth in fish seemed to exist between 1 and 10 mgP/kg.

Researches on the development of the vertebral column were focused mainly on the growth of lumbar and cervical regions. However, none of them were related to the development of costovertebral joints. To our knowledge, the relationship between ribs and thoracic vertebrae has never been addressed. The aims of the presented study are: (1) to trace the formation of processes of vertebral arches and articular surfaces of the vertebral bodies, and (2) to trace the development of the ribs, particularly their joint surfaces. Study subjects are staged human embryos (from the 5th week) from the collection of the Department of Anatomy, Poznan University of Medical Sciences in Poland. Serial sections were stained according to methods allowed for tracing the development of investigated structures. The primordia of costovertebral joints are interzones from which differentiate all structures of synovial joints such as joint surfaces, capsule and cavity. Based on the result of this study, these interzones appeared in the embryonic stages 17 and 18 with approximately 40 to 42 postfertilizational days, and the cavitation within the interzone was observed in some embryos from stage 19 with approximately postfertilizational 44 days.

Purpose: This article aims to provide an overview of how spinal deformities can alter normal spine and thoracic cage growth., Methods: Some of the data presented in this article are gathered from studies performed in 1980 and 1990, and their applicability to populations of different ethnicity, geography or developmental stage has not yet been elucidated. In the present article, older concepts have been integrated with newer scientific data available to give the reader the basis for a better understanding of both normal and abnormal spine and thoracic cage growth., Results: A thorough analysis of different parameters, such as weight, standing and sitting height, body mass index, thoracic perimeter, arm span, T1-S1 spinal segment length, and respiratory function, help the surgeon to choose the best treatment modality. Respiratory problems can develop after a precocious vertebral arthrodesis or as a consequence of pre-existing severe vertebral deformities and can vary in patterns and timing, according to the existing degree of deformity. The varying extent of an experimental arthrodesis also affects differently both growth and thoracopulmonary function., Conclusions: Growth is a succession of acceleration and deceleration phases and a perfect knowledge of normal growth parameters is mandatory to understand the pathologic modifications induced on a growing spine by an early onset spinal deformity. The challenges associated with the growing spine for the surgeon include preservation of the thoracic spine, thoracic cage, and lung growth without reducing spinal motion.

Studies on adult bones have demonstrated age-associated accumulation of histomorphological characteristics that have been used to develop age at death estimation methods. These methods are unsuitable for use with subadult bones because the rapid and extensive turnover and replacement of cortical bone that occurs with growth and development removes any age-associated variables such as osteon numbers. The qualitative analysis of 72 rib samples from individuals of known age has identified a sequence of developmental stages in the growing rib. Recognition of the systematic pattern of changes in the subadult rib has resulted in the development of a four phase qualitative method of age estimation applicable to the subadult rib cortex.

There are few studies that have addressed the effects of prenatal exposure of topiramate on ossification of the bones derived from the paraxial mesoderm. This study aimed to evaluate skeletal ossification of ribs and vertebrae in 20-day-old rat fetuses after maternal exposure to two therapeutic doses of topiramate. Three groups of Sprague-Dawley pregnant rats were used: control, topiramate 50 mg/kg/day and topiramate 100 mg/kg/day treated groups. Topiramate was administered by gavage from day 6-19 of gestation. Fetuses were collected on day 20 by caesarean section. Fetal bones were stained with alizarin red and ossification was assessed. Results showed significant delayed ossification of ribs and vertebrae in topiramate-exposed fetuses at both doses and the effects were not dose dependent. In all examined groups, there was a direct correlation between the fetal weight and the number of complete ossified vertebral centers. Also, there were significant increases in skeletal abnormalities, particularly in ribs in both treated groups when compared to the control group. In conclusion, therapeutic doses of topiramate should be taken cautiously during pregnancy as they lead to fetal growth restriction and increases abnormalities of axial skeleton in rat fetuses.

Background: The Vertical Expandable Prosthetic Titanium Rib (VEPTR™; Synthes North America, West Chester, PA) reportedly controls spinal deformity associated with constrictive chest wall conditions., Questions/purposes: We asked whether spine-to-spine constructs using VEPTR™ instrumentation in combination with standard spinal instrumentation could be deployed to salvage failed rib-to-spine constructs used originally in patients with constricted chest walls and to primarily treat progressive spinal deformity without chest wall abnormalities., Patients and Methods: Fifty patients were treated with VEPTR™ constructs for thoracic insufficiency syndrome at our center between 2001 and 2007. Fourteen of these 50 patients had placement of a spine-to-spine construct using a VEPTR™ implant in combination with standard spinal implants and are the subject of this retrospective review. Five had prior rib-based VEPTR™ or growing implants with an average of two failures before this surgery. Radiographic variables, preceding treatment, complications, and changes in ambulatory status, were recorded. The minimum followup was 2 years (mean, 35 months; range, 2-4 years)., Results: After an average of five expansions in these 14 patients, positive changes were recorded for Cobb angle, T1-S1 height, sagittal balance, and space available for the lung. Complications included two rod fractures, two superficial infections, and one deep infection with rod removal., Conclusions: VEPTR™ instrumentation as a spine-to-spine growing-rod construct demonstrated ease of implantation and expansion, with complication rates similar to other reported devices. This study suggests growing constructs using VEPTR™ can be used with relatively few complications and extends the potential uses of this instrumentation system.

Background: Kyphoscoliosis is considered a relative contraindication to treatment with the Vertical Expandable Prosthetic Titanium Rib (VEPTR(®); Synthes Inc, Paoli, PA). Nevertheless, patients do present with early-onset kyphoscoliosis and thoracic insufficiency syndrome, and no suitable alternative treatments are currently available. However, it is unclear whether VEPTR(®) is reasonable for treating patients with kyphoscoliosis., Questions/purposes: We determined whether VEPTR(®) controls progression in patients with kyphoscoliosis and, if so, what methods might be used to improve control of deformity progression in these patients., Patients and Methods: We retrospectively reviewed 14 patients who had VEPTR(®) treatment of early-onset kyphoscoliosis. Degrees of kyphosis and scoliosis before, during, and after treatment were measured, and levels of instrumentation, thoracic dimensions, and complications were recorded. Minimum followup was 1.7 years (average, 5.8 years; range, 1.7-12.8 years)., Results: While scoliosis was stabilized, kyphosis increased a mean of 22° at last followup. Supple kyphosis became rigid during treatment. Proximal cradle cutout was a recurring problem. Distal anchors placed too proximally had inadequate lever arms to control kyphosis., Conclusions: Progression of kyphosis can be minimized during VEPTR(®) treatment by early extension of the construct to the second ribs bilaterally, distal extension of hybrid constructs to the pelvis, use of bilateral hybrid VEPTR(®) implants, and use of redesigned VEPTR(®) constructs that enhance fixation at the upper end. While our early results suggest these devices control progression of kyphosis, longer followup with more patients will be required to confirm the concept in these patients.

Background: Using a tethering technique, a porcine model of scoliosis has been created. Ideally, tether release before placement and evaluation of corrective therapies would lead to persistent scoliosis., Questions/purposes: Does release of the spinal tether result in persistent deformity?, Methods: Using a unilateral spinal tether and ipsilateral rib cage tethering, scoliosis was initiated on seven pigs. The spinal tether was released after progression to a Cobb angle of 50°. Biweekly radiographs were taken for 18 weeks after tether release to evaluate longitudinal changes in coronal and sagittal Cobb angles. Postmortem fine-cut CT scans were used to evaluate vertebral and disc wedging and axial rotation; results were compared to a previously published data set of 11 animals euthanized before release of the tether (control group)., Results: Radiographic analysis demonstrated two responses to tether release: a persistent deformity group and an autocorrective group. Differences between these two groups included number of days with the tether in place before reaching a Cobb angle of 50° and degree of deformity immediately after scoliosis induction. CT analysis of the tether release versus tether intact groups demonstrated progression in vertebral body wedging without differences in apical rotation., Conclusions: With the appropriate inducing parameters, release of the spinal tether does not systematically result in deformity correction. Tether release resulted in a reduction in Cobb angle in the first several weeks followed by steady curve progression. Deformity progression was confirmed using detailed CT morphometric analysis.

Background: Early-onset scoliosis describes progressive spinal deformity of varying etiologies in the growing child. The management of early-onset scoliosis is challenging, with many treatment options but no conclusive evidence for the best treatment method., Questions/purposes: We describe a bilateral percutaneous rib-to-pelvis technique, present our early experience with this technique in patients with early-onset scoliosis, identify adverse events, and determine whether these are comparable to those for other current techniques., Description of Technique: The VEPTR(®) device is placed through three small incisions that allow for attachment of rib hooks bilaterally at the upper end and through pelvic hooks at the distal end, providing distraction forces to correct the deformity while allowing for growth., Patients and Methods: We retrospectively reviewed all 37 patients with early-onset scoliosis treated with the bilateral rib-to-pelvis VEPTR(®) technique from 2003 and 2009. Patients were evaluated for demographics, diagnosis, curve correction, and adverse events and divided into two groups: ambulatory and nonambulatory. The 18 ambulatory patients underwent 139 procedures and the 19 nonambulatory patients underwent 100 procedures. Average followups were 84 and 64 months in the ambulatory and nonambulatory groups, respectively., Results: The rate of adverse events per procedure was 13%. Thirty-nine percent of ambulatory patients developed a marked crouched gait over time. The rate of adverse events in the nonambulatory group was 15%., Conclusions: This technique appears a reasonable alternative to growing rods for the management of early-onset scoliosis in nonambulatory children due to the low rate of adverse events. Due to the increased incidence of crouched gait, we have abandoned this technique in ambulatory children unless there is no option to attach the distal fixation to the spine.

Multiple Hereditary Exostoses (MHE) syndrome is caused by haploinsufficiency in Golgi-associated heparan sulfate polymerases EXT1 or EXT2 and is characterized by formation of exostoses next to growing long bones and other skeletal elements. Recent mouse studies have indicated that formation of stereotypic exostoses requires a complete loss of Ext expression, suggesting that a similar local loss of EXT function may underlie exostosis formation in patients. To further test this possibility and gain greater insights into pathogenic mechanisms, we created heterozygous Ext1(+/-) and compound Ext1(+/-)/Ext2(+/-) mice. Like Ext2(+/-) mice described previously (Stickens et al. Development 132:5055), Ext1(+/-) mice displayed rib-associated exostosis-like outgrowths only. However, compound heterozygous mice had nearly twice as many outgrowths and, more importantly, displayed stereotypic growth plate-like exostoses along their long bones. Ext1(+/-)Ext2(+/-) exostoses contained very low levels of immuno-detectable heparan sulfate, and Ext1(+/-)Ext2(+/-) chondrocytes, endothelial cells and fibroblasts in vitro produced shortened heparan sulfate chains compared to controls and responded less vigorously to exogenous factors such as FGF-18. We also found that rib outgrowths formed in Ext1(f/+)Col2Cre and Ext1(f/+)Dermo1Cre mice, suggesting that ectopic skeletal tissue can be induced by conditional Ext ablation in local chondrogenic and/or perichondrial cells. The study indicates that formation of stereotypic exostoses requires a significant, but not complete, loss of Ext expression and that exostosis incidence and phenotype are intimately sensitive to, and inversely related to, Ext expression. The data also indicate that the nature and organization of ectopic tissue may be influenced by site-specific anatomical cues and mechanisms., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Thoracic insufficiency syndrome represents a novel form of postnatal restrictive respiratory disease occurring in children with early-onset scoliosis and chest wall anomalies. Expansion thoracoplasty improves lung volumes in children with thoracic insufficiency syndrome; however, how it affects lung development is unknown., Questions/purposes: Using a rabbit model of thoracic insufficiency syndrome, we evaluated the effect of expansion thoracoplasty on the response of biologic mechanisms in the alveolar microstructure., Methods: Using archived material from a previous experiment, 10 4-week-old New Zealand rabbits were divided into three groups: normal (n = 3), disease (n = 3), and treated (n = 4). Left ribs four to eight were tethered in seven rabbits at age 5 weeks to induce hypoplasia of the left hemithorax (disease). At age 10 weeks, four of these rabbits were treated by expansion thoracoplasty (treated). At age 24 weeks, lungs were excised and processed. Alveolar density and parenchymal airspace were measured on histologic sections. Immunohistochemistry was performed for vascular endothelial growth factor receptor 2 (angiogenesis), KI-67 (cell proliferation), and RAM-11 (macrophages)., Results: Alveolar walls were poorly perfused and airspace fraction was larger (emphysematous) in disease rabbits than normal or treated rabbits. Immunohistochemistry provided inconclusive evidence to support the concept that pulmonary hypoplasia is induced by thoracic insufficiency syndrome and controlled by expansion thoracoplasty., Conclusions: Treatment of thoracic insufficiency syndrome by expansion thoracoplasty may prevent emphysematous changes in the alveolar microstructure, thereby enhancing gas exchange.

The development of scoliosis in animal models after inducing asymmetric rib growth suggests the possible role of asymmetric rib growth in the etiopathogenesis of adolescent idiopathic scoliosis (AIS). Asymmetric rib length is well recognized in idiopathic scoliosis; however, whether this rib asymmetry is primary or secondary has not been clearly documented. The objectives of this study were to investigate any rib length asymmetry in patients with AIS and compare those with scoliosis with syringomyelia (SS) with the intention of elucidating any relationship between rib growth and pathogenesis of AIS. Forty-eight AIS and 29 SS with apical vertebrae located between T7 and T9 were recruited. The average age was 13.5 ± 2.3 versus 12.5 ± 3.4 years, and the average Cobb angle of thoracic curve was 43.3° ± 16.4° versus 45.6° ± 22.6° in patients with AIS or SS, respectively. The length of all ribs was measured from the tip of costal head to the end of the same rib by built-in software on spiral computed tomography. At the levels of the apical vertebrae, the vertebrae above and below the apex, the mean discrepancy in rib length (concave minus convex rib) was 7, 4 and 7 mm, respectively, in AIS group (p < 0.01), and 6, 5 and 7 mm in SS group, respectively (p < 0.01). The rib length discrepancy between concave and convex sides was significantly correlated with the magnitude of the Cobb angle of thoracic curve in both AIS and SS groups (p < 0.01). Similar findings of the asymmetry of rib length in both AIS and SS patients pointed strongly to the fact that the rib length asymmetry in apical region is most likely secondary to the scoliosis deformity rather than playing a primary role in the etiopathogenesis.

Disparities in longitudinal growth within a species can be partly explained by endocrinological differences. We hypothesized that regulatory networks acting locally in the growth plate may also be important. We tested this hypothesis by evaluating the IGF/IGFBP expression, the vitamin D pathway, and the PTHrP-Indian hedgehog (IHH) feedback loop in rib growth plates from 10- and 21-wk-old small- (Miniature Poodles, MP) and large-breed dogs (Great Danes, GD) using immunohistochemistry and quantitative (q)PCR. The rib growth plates of GD were 1.7 times thicker compared with those of MP, with larger proliferative (in absolute terms) and larger hypertrophic (in absolute and relative terms) zones. IGF/IGFBP gene expression profiling of the growth plates revealed decreased gene expression of igfbp2, -4, and -6 and an unaltered expression of igf-I and igf-II and their respective receptors in GD vs. MP. Immunohistochemistry and qPCR findings showed that the vitamin D pathway was more active in GD than in MP. Staining for 1α- and 24-hydroxylase was more abundant and intense in GD and the gene expressions of 1α-hydroxylase and the vitamin D receptor-driven 24-hydroxylase were six- and eightfold higher in GD vs. MP, respectively. Consistent with the immunohistochemistry findings, the expression of mRNA for components of the parathyroid hormone-related peptide (PTHrP)-IHH loop was different in GD compared with MP, with there being a relative threefold downregulation of Pthrp and a tenfold upregulation of Ihh in GD vs MP. These differences suggest that the effects of IHH in the regulation of chondrocyte proliferation and hypertrophy, both independently of PTHrP, can become more dominant during rapid growth rates. In conclusion, our data suggest that, in addition to modest endocrine differences, more pronounced changes in the expression of locally acting regulatory networks, such as the IGF system, vitamin D pathway, and PTHrP-IHH feedback loop are important contributors to within-species disparities in growth rates.

Study Design: Morphology comparisons between the pedicle and pedicle rib unit in the immature spine were conducted through computed tomographic scans., Objective: To establish reference data concerning the dimensions of the pedicle rib unit for normal children., Summary of Background Data: Until recently, studies on extrapedicular fixation were mostly based on adults. No published study has assessed the morphology of the pedicle rib unit in the normal immature population. Establishment of the basic morphometric characteristics may be beneficial in treating immature patients with spinal disorders., Methods: Measurements were carried out on the chest computed tomography scans of the immature Chinese patients without any spinal disorder findings. The width, chord length, and transverse angle of the pedicle rib unit were obtained and compared with the corresponding pedicle index. Difference between sex groups was measured, and significant statistical correlations were carefully studied to determine potentially important clinical relationships., Results: The mean values and the standard deviations obtained for each thoracic segment were determined. The width, chord length, and transverse angle of the pedicle rib unit were significantly larger than corresponding pedicle measurements at all levels. Significant difference for measurements between sexes was found at some segments. Compared with the sex, age seemed to have a more significant effect on the measurements. Linear pedicle and pedicle rib unit dimensions had good correlations with age, whereas the transverse angle seemed to change little with age., Conclusion: Compared with the dimensions of the immature pedicle, the significantly larger size of the corresponding pedicle rib unit provides a more ample space, which accommodates screws with larger diameters. Extrapedicular vertebral body fixation was anatomically feasible for the immature spine. The new procedure should be cautiously applied to avoid potential implant failures or a new deformity because of the growth of the immature spine.

Age estimation in the subadult skeleton can be rather precise when the epiphyses and dentition are present, but incomplete or commingled remains still present a challenge. Histomorphometric age-at-death estimation methods developed for use on adults are based on the age-associated accumulation of osteons. In the growing skeleton, there is a poor correlation between osteon numbers and age until the latter half of the second decade. As a result, there has been no histological aging method for use in subadults. The analysis of the rib cortex of 72 subadults ranging in age from 2 to 21 years has identified a series of developmental changes in the bone microstructure that can be used to estimate age. This qualitative method utilizes the systematic changes in rib cortical morphology to classify ribs into one of four age phases. This method can be applied to immature skeletons in forensic, archaeological, and paleontological contexts.

The role of the vertebral body's rotation and the loading conditions of the brace has not been clearly identified in adolescent idiopathic scoliosis. This study aimed to implement a finite element (FE) model of C-type scoliotic spines to investigate the influence of different loading conditions on variations of Cobb's angle and the vertebral rotation. The scoliotic FE model was constructed from C7 to L5, and its geometry was the right thoracic type (37.4°) with an apex over T7. Three loading conditions included a medial-lateral (ML) and anteroposterior (AP) force with a magnitudes of 100-0, 80-20 and 60-40 N. Those forces were respectively applied over the 6th, 7th and 8th ribs. According to an analysis of Cobb's angle, the 100 N ML force that was applied over the 8th rib could achieve the best correction effect. Furthermore, the ML force was dominant in alterations of Cobb's angle, whereas the AP force was dominant in alterations of the axial vertebral rotation. Additionally, the level below the apex was the most appropriate level to apply the force to correct C-type scoliosis.

The development of scoliosis in animal models with induced asymmetric rib growth suggested the possible role of asymmetric rib growth in the etiopathogenesis of adolescent idiopathic scoliosis (AIS). Asymmetric rib length is well recognized in idiopathic scoliosis, however, whether this rib asymmetry was primary or secondary has not been clearly documented. We measured the lengths of all ribs in 48 patients with AIS and 29 patients with scoliosis associated with syringomyelia and Chiari I malformation (SS). In both groups, ribs on the concave side were significantly longer than those on the convex side in apical area. The rib length difference was significantly associated with magnitude of the Cobb angle of thoracic curve. However, the rib length discrepancy showed no difference between AIS and SS group. The finding of similar asymmetry of rib length in both AIS and SS patients with thoracic curve indicated the discrepancy found in AIS might be secondary to the spinal deformity rather than its etiological factor.

Object: Few options exist for the treatment of severe, early onset scoliosis. Goals of treatment include stabilizing curve progression while allowing for normal spine, chest, and lung growth. The vertical expandable prosthetic titanium rib (VEPTR) is a novel device designed to control the spine deformity while permitting lung and spine growth. In this paper the authors report their experience with using bilateral VEPTRs from the ribs to the pelvis for children with severe, early onset scoliosis., Methods: Eleven children were identified who had been treated with bilateral VEPTRs from the ribs to the pelvis. The authors conducted a retrospective review and collected the following data: clinical diagnosis, age at surgery, number of lengthening procedures, and complications. In addition, pre- and postoperative radiographs were reviewed to measure maximum Cobb angle (both thoracic and lumbar), thoracic height, total spine height as measured from T-1 to S-1, thoracic kyphosis (T2-12), and lumbar lordosis (L1-S1)., Results: The average patient age at surgery was 71 months; the mean preoperative thoracic Cobb angle was 81.7 degrees . This angle was corrected to 50.6 degrees immediately postoperatively, and this correction was maintained; at the most recent follow-up the curves averaged 58 degrees . Similarly, the preoperative kyphosis (T2-12) angle measured 43 degrees preoperatively, 23 degrees immediately postoperatively, and 37 degrees at the most recent follow-up evaluation. The patients underwent a total of 41 lengthening procedures (average 3.7 lengthening procedures per patient), and overall spine length increased from 23.1 cm preoperatively, to 27.3 cm immediately postoperatively, to 29.4 cm at the final follow-up (an average of 25 months). Four (36.4%) of the 11 patients experienced complications., Conclusions: The VEPTR offers a viable treatment option for children with severe, early onset scoliosis. It achieves and maintains spinal deformity correction, while allowing for continued spine and chest-wall growth. Complication rates are similar to those reported for other growing systems.

Purpose: The present investigation was designed to study the modulation of abdomen and rib cage movements during vocalization over a period of development associated with rapid decreases in the compliance of the chest wall., Method: Rib cage and abdominal kinematics were recorded during spontaneous vocalizations in 7- and 11-month old infants. Principal component analysis was used to represent each infant's abdomen and rib cage traces as the weighted sum of a small number of principal component (PC) waveforms., Results: The fundamental periods of infants' PC waveforms in the 11-month group were significantly shorter than those in the 7-month group. In addition, the variance contributed by PCs describing unidirectional patterns of respiratory movement decreased in the 11-month group, whereas the variances contributed by PCs describing modulated patterns of movement increased. Lastly, the extent to which abdomen and rib cage movements predicted the duration of corresponding vocalizations also increased significantly in the 11-month group compared with the 7-month group., Conclusions: The findings of the present study were consistent with the hypothesis that decreases in the compliance of the chest wall result in more rapid modulation of chest wall movements and greater control of those movements by the developing neuromuscular system.

Uncinate processes extend off the vertebral ribs in most species of bird. The processes are a crucial component of ventilatory mechanics, being involved in inspiration and expiration. Here we examine the pattern of ossification of the uncinate processes using histochemistry and biomechanical testing in developing domestic turkeys (Meleagris gallopavo). Ossification begins just before hatching, and the processes are fully ossified in the adult bird. We suggest that the development of these processes is linked to the onset of air breathing and the increase in sternal mass that occurs after hatching.

Cartilage and bone are formed into a remarkable range of shapes and sizes that underlie many anatomical adaptations to different lifestyles in vertebrates. Although the morphological blueprints for individual cartilage and bony structures must somehow be encoded in the genome, we currently know little about the detailed genomic mechanisms that direct precise growth patterns for particular bones. We have carried out large-scale enhancer surveys to identify the regulatory architecture controlling developmental expression of the mouse Bmp5 gene, which encodes a secreted signaling molecule required for normal morphology of specific skeletal features. Although Bmp5 is expressed in many skeletal precursors, different enhancers control expression in individual bones. Remarkably, we show here that different enhancers also exist for highly restricted spatial subdomains along the surface of individual skeletal structures, including ribs and nasal cartilages. Transgenic, null, and regulatory mutations confirm that these anatomy-specific sequences are sufficient to trigger local changes in skeletal morphology and are required for establishing normal growth rates on separate bone surfaces. Our findings suggest that individual bones are composite structures whose detailed growth patterns are built from many smaller lineage and gene expression domains. Individual enhancers in BMP genes provide a genomic mechanism for controlling precise growth domains in particular cartilages and bones, making it possible to separately regulate skeletal anatomy at highly specific locations in the body., Competing Interests: The authors have declared that no competing interests exist.

Although we are starting to understand the molecular basis of shell development based on the study of cryptodires, basic comparative ontogenetic data for the other major clade of living turtle, the pleurodires, are largely missing. Herein, the developmental and phylogenetic relation between the bony shell and endoskeleton of Pleurodira are examined by studying histological serial sections of nine specimens of three different species, including an ontogenetic series of Emydura subglobosa. Emphasis is given to the portion of the carapace in which ribs and vertebral spinous processes become part of the carapace. Central questions are how neurals and costals are formed in pleurodiran turtles, whether costals and neurals are of endoskeletal or exoskeletal origin, and what ontogenetic factors relate to neural reduction of some Pleurodira. The neurals and costals do not develop as independent ossification centers, but they are initial outgrowths of the periosteal collar of endoskeletal ribs and neural arches. Slightly later in development, the ossification of both shell elements continues without a distinct periosteum but by metaplastically ossifying precondensed soft-tissue integumentary structures. Through ontogeny, ribs of the turtles studied are closely associated with the hypaxial intercostalis musculature while epaxial interspinalis musculature connects the neural arches. We here propose an alternative structural hypothesis for the neural reduction and, ultimately, the complete loss of the neural series. The complete reduction of neurals in Emydura spp. may be linked to heterochrony, accompanied by a restricted influence of epaxial musculature and epidermal-dermal interaction in shell bone formation., ((c) 2008 Wiley-Liss, Inc.)

A descriptive clinical study in healthy adolescents was done to evaluate the clinical shoulder balance and analyze the correlation between clinical and radiological parameters which are currently used to evaluate shoulder balance. In addition to trunk shift and rib hump, shoulder balance is one of the criteria that are used to evaluate the outcomes in spinal deformity surgery. Several methods have been proposed to evaluate the shoulder balance in scoliotic patients; however, there is no uniformity to these methods in the current literature. Patients who applied to pediatric clinic without musculoskeletal pathology formed the patient population. Volunteers were asked to fill out a questionnaire assessing shoulder balance perception and had their clinical photograph taken simultaneously with a P-A chest X-ray. The clinical shoulder balance was evaluated through analysis of the clinical photograph. The X-rays were used to evaluate the radiological shoulder balance. The evaluated parameters included coracoid height difference (CHD), clavicular angle (CA), the clavicle-rib cage intersection difference (CRID), clavicular tilt angle difference (CTAD), and T1-tilt. The study group was composed of 48 male and 43 female patients with an average age of 13.6 +/- 2.1 (10-18) years. In the questionnaire, all patients stated that their shoulders were level. The digital photographs revealed that only 17(18.7%) adolescents had absolutely level shoulders. The average height difference between shoulders was 7.5 +/- 5.8 mm. The average CHD was 6.9 +/- 5.8 mm, average CA was 2.2 +/- 1.7 degrees , average CRID was 4.8 +/- 3.6 mm, average CTAD was 4 +/- 3.2 degrees , and average T1-tilt was 1.3 +/- 1.4 degrees . CHD, CA, and CRID demonstrated high correlation with clinical pictures, whereas CTAD demonstrated moderate and T1-tilt demonstrated only mild correlation. The radiological parameters used to evaluate the shoulder balance correlate with the clinical appearance. Contrary to popular belief, shoulder balance in healthy adolescents often does not exist.

We report the case of a 3-year-old girl with temporomandibular ankylosis, which was treated with a costochondral graft and required two further resections because the grafted tissue had overgrown.

Study Design: This study was designed to investigate the difference of rib length of idiopathic scoliosis using experimentally induced scoliosis by pinealectomy in chickens., Objective: To find whether the difference of rib length may play a role for the development of scoliosis in pinealectomized chickens., Summary of Background Data: In experimental and clinical studies, asymmetric growth of the rib has been considered to be one of factors for the development of idiopathic scoliosis. Pinealectomy in chickens consistently produces scoliosis with lordosis, vertebral rotations, and rib humps, which have anatomic characteristics similar to those of human idiopathic scoliosis., Methods: A total of 30 chickens were divided into two groups: pinealectomy in 15 chickens and sham operation in 15 chickens served as control. The surgeries were performed on the second day after hatching. The chickens in both groups were killed at 3 months of age. The spines were examined radiologically for the presence of a scoliotic curve. Also, the ribs at each level were measured and compared between left and right sides., Results: All pinealectomized chickens that developed scoliosis showed rib humps. In contrast, none of chickens in a sham operation developed scoliosis. In both groups, there were no significant asymmetries in the rib length., Conclusion: Our results indicated that rib length was not related to the experimental scoliosis examined in this study. Further study is encouraged to investigate if the asymmetric rib length plays a role in causing or promoting other types of scoliosis, especially in primate or human idiopathic scoliosis.

Supernumerary or accessory ribs (SNR), either lumbar (LR) or cervical (CR), are a common finding in standard developmental toxicology bioassays. The biological significance of these anomalies within the regulatory arena has been problematic and the subject of some debate. In rodents, the spontaneous incidence of SNR is species and strain related and ranges from <1% to >30%. Compound-induced LR are induced by a wide variety of chemical and physical agents when pregnant animals are exposed during specific gestational periods. A significant portion of the agent-induced LR may be due to maternal factors, as it has been shown that stress alone will induce LR in rodents. SNR are not isolated phenomena and signify basic alterations in the architecture of the axial skeleton. LR are associated with longer ribs, increased numbers of vertebrosternal ribs, and the presence of extra presacral vertebrae ("anteriorization"). CR are associated with reduced numbers of vertebrosternal ribs (posteriorization). It is evident that SNR are not a single anomaly, but consist of two unrelated structures: an extra rib that has a cartilaginous segment at the distal end, and an ossification site that lacks cartilage. These have a bimodal size distribution, with the population of extra ribs being significantly longer than the ossification sites, and 0.6 mm can be used as an approximate length for distinguishing the two populations in mice. Extra ribs are permanent structures in contrast to ossification sites that disappear postnatally, probably becoming part of the lateral transverse vertebral processes. SNR are also found in humans although, in contrast to laboratory species, CR are more commonly noted. SNR are associated with adverse heath effects, and CR with inducing thoracic outlet disease characterized by diminished blood flow and altered position of the ganglia and nerve roots in the area of the C7-T1 vertebrae. LR are associated with lower back pain and L4-5 degeneration. The incidence of CR is greatly reduced in adult humans as compared to fetuses, and it has been hypothesized that fetal "SNR" may be largely composed of ossification sites that disappear postnatally. The mechanisms involved in the formation of extra ribs are not understood at this time, although the fact that the early sensitive periods for their initiation during embryogenesis is coupled with the associated changes in the axial skeleton argues for their induction being due to fundamental changes in gene expression. The sum of the experimental evidence supports the idea of SNR being composed of two different structures: extra ribs that are permanent dysmorphological structures that may be induced by xenobiotics and/or maternal stress, and ossification sites that may be transient variations in the formation of the lateral processes of the vertebrae.