Search

Objectives: The breathing phase for the determination of thoracic indices in patients with pectus excavatum is not standardized. The aim of this study was to identify the best period for reliable assessments of morphologic indices by dynamic observations of the chest wall using real-time MRI. Methods: In this prospective study, patients with pectus excavatum underwent morphologic evaluation by real-time MRI at 3 T between January 2020 and June 2021. The Haller index (HI), correction index (CI), modified asymmetry index (AI), and modified eccentricity index (EI) were determined during free, quiet, and forced breathing respectively. Breathing-related differences in the thoracic indices were analyzed with the Wilcoxon signed-rank test. Motion of the anterior chest wall was analyzed as well. Results: A total of 56 patients (11 females and 45 males, median age 15.4 years, interquartile range 14.3–16.9) were included. In quiet expiration, the median HI in the cohort equaled 5.7 (4.5–7.2). The median absolute differences (Δ) in the thoracic indices between peak inspiration and peak expiration were ΔHI = 1.1 (0.7–1.6, p <.001), ΔCI = 4.8% (1.3–7.5%, p <.001), ΔAI = 3.0% (1.0–5.0%, p <.001), and ΔEI = 8.0% (3.0–14.0%, p <.05). The indices varied significantly during different inspiratory phases, but not during expiration (p >.05 each). Furthermore, the dynamic evaluation revealed three distinctive movement patterns of the funnel chest. Conclusions: Real-time MRI reveals patterns of chest wall motion and indicate that thoracic indices of pectus excavatum should be assessed in the end-expiratory phase of quiet expiration. Key Points: • The thoracic indices in patients with pectus excavatum depend on the breathing phase. • Quiet expiration represents the best breathing phase for determining thoracic indices. • Real-time MRI can identify different chest wall motion patterns in pectus excavatum. [ABSTRACT FROM AUTHOR]

Introduction: Rib anomalies include numerical and structural categories. This study investigates the costal abnormalities by computed tomography scan in an Iranian population Methods: In a retrospective study, 400 Chest CT scans (225 (56.2%) male, and 175 (43.8%) female) aging from 18 to 89 were investigated for one year (2020 to 2021). Images were carefully observed and various abnormalities such as cervical ribs, elongated C7 transverse processes, bridging ribs, and bifid ribs were reported. Results: Among 400 chest CT scans investigated for this study, 375 (93.7%) cases were normal and 25 (6.3%) cases were abnormal. Fourteen (8% of all the female patients) and eleven (4.9% of all the male patients) participants of abnormal cases were female, and male, respectively. Among abnormal cases, four cases of bilateral cervical ribs (total prevalence (TP)=1%), four cases of bilateral elongated C7 transverse process (TP=1%), four cases of bridging first and second ribs on the left side (TP=1%), twelve cases of bifid ribs on the left (TP=3%), and one uncommon male case were reported. Conclusion: Being aware of the common abnormalities of rib cage is helpful in differentiating the diagnosis and seeking for possible other accompanied diseases such as thoracic outlet or Gorlin syndrome. In this study, twenty five cases of abnormal rib morphology were reported. [ABSTRACT FROM AUTHOR]

Objective: To determine associations between fetal rib number anomalies detected on ultrasonography and chromosomal anomalies and other structural anomalies, and the outcome of affected pregnancies. Methods: All cases of fetal rib number anomalies referred to the Prenatal Diagnosis Clinic of Queen Elizabeth Hospital between 1 January 2016 and 31 December 2019 were reviewed. Fetal ribs were examined by static threedimensional multiplanar or volume contrast ultrasonography. Genetic counselling was offered. The prenatal and postnatal records were reviewed. Results: 21 fetuses with rib number anomalies were identified over 4 years. The most common presentation was unilateral or bilateral absence of the 12th thoracic rib (n=12, 57.1%), followed by the presence of lumbar rib (n=6, 28.6%) and the presence of cervical rib (n=3, 14.3%). Three (14.3%) fetuses were identified to have anomalies in other systems: unilateral absence of nasal bone (n=1) and minor vascular anomalies (n=2). One patient with multiple anomalies of the fetus underwent amniocentesis, and the chromosomal microarray analysis was normal. Postnatally, 13 babies had chest radiographs taken. Two were confirmed to have normal number of ribs. Prenatal and postnatal findings were consistent in 6 (46.2%) babies. Conclusion: Fetal rib number anomalies were an isolated finding in most cases. The prognosis is good in the absence of other major anomalies. The accuracy of prenatal ultrasonography appears to be low. These findings do not support routine counting of fetal rib number in second-trimester ultrasonography. [ABSTRACT FROM AUTHOR]

Campomelic dysplasia (CD) is a rare form of skeletal dysplasia (incidence 1:200,000 births) which is associated with characteristic phenotypes including bowing of the limbs, a narrow thoracic cage, 11 pairs of ribs, hypoplastic scapulae, macrocephaly, flattened supraorbital ridges and nasal bridge, cleft palate, and micrognathia. In addition to the skeletal abnormalities, hydrocephalus, hydronephrosis, and congenital heart disease have been reported. We describe a preterm neonate who presented with respiratory failure and clinical features of CD. Our case had only 10 pairs of ribs, and to the best of our knowledge this is the first case report of CD with 10 pairs of ribs. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to show the difference between the pulmonary function of children with mild or severe types of pectus excavatum (PE) and post-operative changes in their predicted vital capacity (%VC) following Nuss procedure.Patients and Methods: One hundred and twenty-four of the 208 children who underwent Nuss procedure for PE between January 2007 and March 2016 were deemed eligible for this study and evaluated retrospectively. A spirometry was performed on these children on four occasions: before operation; pre-bar removal; and 1 month and 1 year after bar removal. Ninety-seven of the children who were more than 7 years of age were divided using the Haller Index (HI) into a mild group (n = 54) and a severe group (n = 43) and compared. The children were also divided into three groups based on their age and their %VC was compared at each follow-up occasion.Results: The severe group showed a significantly lower %VC and peak expiratory flow rate than the mild group. %VC change after bar removal showed significantly lower in group aged 11 or over. 43 of the children had spirometry data recorded 1 year after bar removal which, compared with 1 month after bar removal, showed a significant higher %VC in groups aged of 10 or under.Conclusion: Nuss procedure in children aged of 10 or under proved to be an advantage in the post-operative pulmonary function. [ABSTRACT FROM AUTHOR]

Introduction: Vertical scapular osteotomy (VSO) is a known procedure for Sprengel's shoulder deformity. Its surgical results in the presence of rib cage anomalies largely remain unreported.Material and Methods: The influence of simple and complex rib anomalies on shoulder abduction, Cavendish grade, and radiological Rigault's score following VSO was studied retrospectively in eight paediatric patients. The omovertebral bar effect on surgical outcome was also observed.Results: The average age at surgery was 6.8 years. Rib anomalies were found in seven patients among which four had simple (50%) and three complex rib anomalies (37.5%). At mean post-surgery follow-up of four years, the overall average gain in shoulder abduction was 21°. In patients having either no or simple rib anomalies (n = 5), the mean pre-operative abduction and follow-up increase was more (132°; gain 28°) compared to complex rib anomalies (110°; gain 10°). The overall average pre-operative Cavendish score changed from 2.8 to 1.5. The correction was more complete in simple or no rib anomalies. The mean preoperative Rigault score was 2.25 with only a small improvement (0.38) noted in follow-up. Two patients with omovertebral bar attained good cosmetic and functional results.Conclusions: The current study was at variance with the previously published studies in terms of improved shoulder abduction following VSO especially complex rib anomalies. The omovertebral bar was not an adverse prognosticating factor. [ABSTRACT FROM AUTHOR]

Patients with pectus excavatum (PE) frequently present with complaints of exercise intolerance and cardiopulmonary symptoms. There continues to be controversy regarding the physiologic benefits of repair. The aim of this review is to summarize and discuss recent data regarding the cardiopulmonary effects of PE deformity and the evidence for improvement obtained after surgical repair including (1) a greater efficiency of breathing (chest wall mechanics), (2) improvement in pulmonary restrictive deficits, (3) an increase in cardiac chamber size and output, with improved cardiac strain and strain rate, and (4) improvement in exercise capacity. [ABSTRACT FROM AUTHOR]

Purpose: Jarcho-Levin syndrome patients commonly suffer from repeated respiratory infections and become ventilator dependent due to an insufficient thoracic volume. Multiple congenital vertebral anomalies are associated with this genetic disorder and results in stunted spine growth. The purpose of this case report is to present the results of a hybrid vertical expandable prosthetic titanium rib (VEPTR) and magnetically controlled growing rod (MCGR) construct for the management of a patient with Jarcho-Levin syndrome.Methods: A boy with Jarcho-Levin syndrome undergoes a hybrid VEPTR-MCGR construct to treat his thoracic insufficiency syndrome and spinal deformity.Results: The patient could wean off ventilator and had reduced chest complications with the construct. He was also able to achieve some spine length gain with the distraction device. However, there were limitations in prolonged treatment as no spine height was gained once he reached 12 years old. Once no further growth can be achieved, the MCGR led to progressive kyphosis. Nevertheless, at latest follow-up after the removal of all implants, the patient could maintain satisfactory correction of both thoracic and spine deformities.Conclusions: A hybrid construct is necessary for managing Jarcho-Levin syndrome as the VEPTR deals with the thoracic cage deformity while the MCGR deals with the spine deformity. [ABSTRACT FROM AUTHOR]

Objective: The authors present a multicenter retrospective series of different benign rib lesions in children operated on using thoracoscopy.Materials and Methods: Between 2005 and 2015, 17 rib resections were performed thoracoscopically, in four French departments of pediatric surgery. Of these 17 cases, 13 exostoses, 2 endochondromas, 1 synostosis, and 1 Cyriax's syndrome were noted. Inclusion criteria were benign tumors or rib anomalies such as synostosis, in children younger than the age of 18 years, and thoracoscopy. Open surgery and malignant tumors were excluded. Thoracoscopy was put forward using one optical port as well as one or two operative ports.Results: Ten patients presented with chest pain, dyspnea, or unexplained cough. Six tumors were incidentally diagnosed. One patient presented with a chest wall deformation. Single-lung ventilation was required in 2 cases. In 1 case of endochondroma, a segmental rib resection was performed, leaving a part of the periosteum and the intercostal vessels and nerve. In this case, rib resection was performed using an endoscopic shaver drill system. As for the other cases, a simple resection of the tumor or of the bridge between two ribs (synostosis) was performed. In these cases, a Codman Kerrison laminectomy rongeur was used. There was no complication during or after surgery. Nevertheless, 2 years after surgery, pain did not disappear in 1 case, probably due to a definitive intercostal nerve damage.Conclusion: Benign rib lesions in children are rare. Thoracoscopy may be offered to reduce the functional deleterious consequences of an open surgery. It may be put forward especially in case of hereditary multiple exostoses where redo procedures may be required. [ABSTRACT FROM AUTHOR]

The secondary flows generated by the rib and the U-shaped bend play a vital role in heat transfer enhancement in two-pass ribbed channels. This paper focuses on the combined effects of rib orientations (N-type and P-type) and rib angles (from 30º to 75º) on flow and heat transfer in two-pass ribbed channels. The 3D steady Reynolds-averaged Navier-Stokes equations coupled with SST k- ω model are solved by using commercial software ANSYS CFX. The CFD model has been validated by experimental data with a good agreement. In addition, the comprehensive evolution of secondary flows and its effect on heat transfer are analyzed in detail by using vortex core technology. The results indicate that the key factor for local heat transfer enhancement is to transport more secondary flows into downstream passage without weakening local secondary flow and energy dissipation of upstream secondary flow. Furthermore, the rib orientation in passage 2 should consider the influence of rib angle so that the local main flow secondary flow has the same rotational direction with the most powerful upstream secondary flow to reduce the energy loss and speed up the growth of local main flow secondary flow as much as possible. [ABSTRACT FROM AUTHOR]

Background: Vertebrae, ribs, and spinal cord are anatomically adjacent structures, and their close relationships are clinically important for planning better corrective surgical approach. The objective is to identify the radiographic characteristics in surgical patients with congenital scoliosis (CS) and coexisting split cord malformation (SCM).Methods: A total of 266 patients with CS and SCM underwent surgical treatment at our hospital between May 2000 and December 2015 was retrospectively identified. The demographic distribution and radiographic data were collected to investigate the characteristics of spine curve, vertebral, rib, and intraspinal anomalies. According to Pang's classification, all patients were divided into two groups: type I group is defined as two hemicords, each within a separate dural tube separated by a bony or cartilaginous medial spur, while type II group is defined as two hemicords within a single dural tube separated by a nonrigid fibrous septum.Results: There were 104 patients (39.1%) in Type I group and 162 patients (60.9%) in Type II group. SCM was most commonly found in the lower thoracic and lumbar regions. The mean length of the septum in Type I SCM was significantly shorter than Type II SCM (2.7 vs. 5.2 segments). Patients in Type I group had a higher proportion of kyphotic deformity (22.1%). The vertebral deformities were simple in only 16.5% and multiple in 83.5% of 266 cases. Patients in Type I group presented higher prevalence of multiple (90.4%) and extensive (5.1 segments) malformation of vertebrae. In addition, hypertrophic lamina and bulbous spinous processes were more frequent in Type I group (29.7%), even developing into the "volcano-shape" deformities. Rib anomalies occurred in 62.8% of all patients and 46.1% of them were complex anomalies. The overall prevalence of other intraspinal anomalies was 42.9%. The most common coexisting intraspinal anomalies was syringomyelia (30.5%).Conclusion: The current study, with the largest cohort to date, demonstrated that patients with CS and coexisting SCM presented high prevalence of multiple vertebral deformities, rib and intraspinal anomalies. The length of the split segment in Type I SCM was shorter than that in Type II SCM. Compared with Type II SCM, patients with Type I SCM presented with higher incidence of kyphotic deformity, more extensive and complicated vertebral anomalies, and more complex rib anomalies. [ABSTRACT FROM AUTHOR]

Background: Thoracoabdominal asynchrony is the nonparallel motion of the ribcage and abdomen. It is estimated by using respiratory inductive plethysmography and, recently, using optoelectronic plethysmography; however the agreement of measurements between these 2 techniques is unknown. Therefore, the present study compared respiratory inductive plethysmography with optoelectronic plethysmography for measuring thoracoabdominal asynchrony to see if the measurements were similar or different. Methods: 27 individuals (9 healthy subjects, 9 patients with interstitial lung disease, and 9 with chronic obstructive pulmonary disease performed 2 cycle ergometer tests with respiratory inductive plethysmography or optoelectronic plethysmography in a random order. Thoracoabdominal asynchrony was evaluated at rest, and at 50% and 75% of maximal workload between the superior ribcage and abdomen using a phase angle. Results: Thoracoabdominal asynchrony values were very similar in both approaches not only at rest but also with exercise, with no statistical difference. There was a good correlation between the methods and the Phase angle values were within the limits of agreement in the Bland-Altman analysis. Conclusion: Thoracoabdominal asynchrony measured by optoelectronic plethysmography and respiratory inductive plethysmography results in similar values and has a satisfactory agreement at rest and even for different exercise intensities in these groups. [ABSTRACT FROM AUTHOR]

Background/aim: Pectus excavatum and pectus carinatum are the most commonly seen anterior chest wall deformities. Recent studies reveal that minimal invasive repair of pectus deformities improves the quality of life. Our aim is to assess the psychosocial functioning and sociodemographic characteristics of pediatric patients with pectus deformities and evaluate the differences between patients operated on with minimal invasive repair techniques and nonoperated patients. Materials and methods: Thirty-two patients with pectus deformities who were operated on 6 months or more before and 31 nonoperated patients participated in the study. The Children's Depression Inventory, Piers-Harris Children's Self-Concept Scale, Capa Social Phobia Scale for Children and Adolescents, Strengths and Difficulties Questionnaire - Self-Report Version (SDQ-SR), and State-Trait Anxiety Inventory for Children - Trait Version were completed by the patients. The SDQ-Parent Report Version (SDQ-PR) was completed by their parents. Results: There were no statistically significant differences between operated and nonoperated patient groups in terms of total scores on the psychiatric rating scales. Prosocial behavior subscale scores in SDQ-SR (P = 0.013) and SDQ-PR (P = 0.019) were lower in the operated group. Conclusion: Prosocial behavior levels were lower in the operated group. Further exploration of the psychosocial profile of pediatric patients with pectus deformities would better elucidate their needs in the course of their socioemotional development. [ABSTRACT FROM AUTHOR]

Background: Skeletal ciliopathies comprise a spectrum of ciliary malfunction disorders that have a profound effect on the skeleton. Most common among these disorders is short rib polydactyly syndrome (SRPS), a recessively inherited perinatal lethal condition characterized by a long narrow chest, markedly shortened long bones, polydactyly and, often, multi-organ system involvement. SRPS shows extensive locus heterogeneity with mutations in genes encoding proteins that participate in cilia formation and/or function. Results: Herein we describe mutations in IFT43, a satellite member of the retrograde IFT-A complex, that produce a form of SRPS with unusual bending of the ribs and appendicular bones. These newly described IFT43 mutations disrupted cilia formation, produced abnormalities in cartilage growth plate architecture thus contributing to altered endochondral ossifcation. We further show that the IFT43 SRPS phenotype is similar to SRPS resulting from mutations in the gene encoding IFT121 (WDR35), a direct interactor with IFT43. Conclusions: This study defnes a new IFT43-associated phenotype, identifying an additional locus for SRPS. The data demonstrate that IFT43 is essential for ciliogenesis and that the mutations disrupted the orderly proliferation and diferentiation of growth plate chondrocytes, resulting in a severe effect on endochondral ossifcation and mineralization. Phenotypic similarities with SRPS cases resulting from mutations in the gene encoding the IFT43 direct interacting protein IFT121 suggests that similar mechanisms may be disrupted by defects in these two IFT-A satellite interactors. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this report is to identify the radiological findings of unilateral tuberculous lung destruction (UTLD). Materials and methods: Thirteen patients with (UTLD) were reviewed from 1999 to 2014. Only patients with radiological evidence of absence of pulmonary parenchyma preserved were included. Clinical and demographic data were obtained and radiological studies (chest radiograph and CT) were retrospectively reviewed. Results: The left lung was more commonly involved (85%). The following radiological findings were found in all cases: a decrease in the diameter of the pulmonary vessels of the affected lung, herniation of the contralateral lung and hypertrophy of the ribs and/or thickening of extrapleural fat. Two radiological patterns were identified: UTLD with cystic bronchiectasis (85%) and UTLD without residual cystic bronchiectasis (15%). Forty-six per cent of cases had respiratory infection symptoms with presence of air-fluid levels in the affected lung as the most common finding in these patients. Conclusions: Total unilateral post-tuberculous lung destruction is an irreversible complication with the following main radiological features: predominantly left-sided location, decreases in the diameter of the ipsilateral pulmonary vessels, herniation of the contralateral lung and hypertrophy of the ribs and/or thickening of extrapleural fat. Teaching Points: • Unilateral tuberculous lung destruction is an irreversible complication of tuberculosis. • Left-side predominance and herniation of the contralateral lung are characteristic. • Decreased diameter of the ipsilateral pulmonary vessels occurred in all patients. • The pattern with residual cystic bronchiectasis is the most frequent. • Superimposed non-tuberculous infections may affect the destroyed lung. [ABSTRACT FROM AUTHOR]

The estimation of age at death is one of the most fundamental biological parameters, determined on skeletal remains in anthropological context. That is why, there is a constant need to improve applied methods. Histomorphometry, which uses microscopic analysis of bone tissue is suggested to be one alternative method. In general, this technique is based on measurements and the determination of the number and density of basic bone structural units, osteons. Osteon density is found to be related with age of the individual. The main goal of this research was to compare results of determined age at death, on the basis of ribs histology, comes from methods proposed by different authors. We analyzed ground cross sections of ribs from archeological origin. The presented methodology is simple in use and effective. Four different methods were tested (Stout and Paine 1992; Cho et al. 2002; Kim et al. 2007; Bednarek et al. 2009). The obtained age results were compared with each other as well as related to the age estimated by standard macroscopic method used in anthropology. Bednarek's method is recognized to be the most supportive for anthropological analyzes. Methodological issues connected with grinding methodology and results interpretation are also presented. Hypothesis about interpopulation as well as histological and dimorphic differences were confirmed. [ABSTRACT FROM AUTHOR]

We retrospectively studied a group of 39 patients admitted in the "Marie Curie" Emergency Pediatrics Hospital of Bucharest in a period of 10 years, suffering from congenital disorders of the thorax. Out of this large group, 32 patient were admitted with Pectus Excavatum. The goal of our study is to demonstrate the advantages of minimally invasive surgery for this type of malformation. We included 39 patients in our study, ages from 0- 24 years old, diagnosed with chest malformations, of which 32 had pectus excavatum associated or not with other pathologies. Minimally invasive surgery was the main choice with great results. Most of the patients had no symptoms, therefor they sought medical care for esthetic reasons. Minimally invasive surgery is efficient, with small complication rate, proving to be the best option. [ABSTRACT FROM AUTHOR]

Bifid or bifurcated ribs are a rare anatomical anomaly that accounts for approximately 28% of known rib abnormalities. Bifurcation always occurs at the sternal end of the rib and its two extremities are joined to a bifid costal cartilage. The presence of bifid rib is frequently accompanied by other anomalies, although this does not necessarily imply that it cannot occur as an isolated anomaly. The clinical significance of the latter has not been established. There have not been many studies done to investigate bifid rib closely aside from individual case reports, presumably due to the condition's extremely low incidence. However the detection of bifid ribs, though mostly incidental, has been proven advantageous for an early and well-rounded diagnosis It has also revealed significant preventive benefits especially in the pediatric patient population. This review is written to provide a comprehensive summary of what is known about bifid ribs and incorporate substantial findings from clinical cases that have been reported in past literature. [ABSTRACT FROM AUTHOR]

A familiarity with the anatomy of some types of bone anomalies is necessary for clinicians involved in many medical areas. The aim of this paper is to review the newest literature concerning the morphology, embryology, clinical image, and therapeutic methods of the cervical ribs in the humans. The incidence of cervical ribs has been found to vary from 0.58% in Malaysian population to 6.2% in Turkish population. Cervical ribs have clinical implications that are generally divided into neurological or vascular. This study is of particular importance for clinicians, as early identification of cervical ribs may prevent life-threatening complications. [ABSTRACT FROM AUTHOR]

Spondylocostal dysostosis (SCD) is a rare anomaly of axial skeleton due to flawed embryological development characterized by malformed ribs, fused ribs, and hemivertebra. Case one: A newborn baby boy with right-sided polythelia, meningocele, fan-like configuration of 3rd and 4th rib, fused 5th and 6th rib, and 3rd thoracic hemivertebra was delivered to a second gravid mother by elective cesarean section. Based on clinical examination and radiological picture, a diagnosis of SCD with meningocele with right-sided polythelia was made. Case two: Baby girl born to primigravida mother by cesarean section had hemivertebrae with multiple right-sided rib defects, short neck, preauricular ear tags, imperforate anus, and rectovaginal fistula. Very few cases of SCD have been reported in literature with neural tube defects (NTDs) with supernumerary nipple, none from developing country. Till now, there is limited literature regarding the associations of SCD none showing its association with the genitourinary system without NTDs. We need to study the causal associations of SCD with detailed genetic work up as it is not so uncommon as presumed. [ABSTRACT FROM AUTHOR]

Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. One treatment to address the thoracic deformities associated with the syndrome is placement of a vertical expandable prosthetic titanium rib. The goal is to restore thoracic volume, symmetry, and function and to allow for growth of the thorax in skeletally immature children. The main topics include indications for placement of a titanium rib, postoperative nursing care, potential complications, long-term follow-up, quality-of-life issues, and implications for critical care nurses and advanced practice nurses. [ABSTRACT FROM AUTHOR]

Introduction: Respiratory failure is the leading cause of mortality in individuals with congenital spine and rib deformities. We present a case report of a child with Jeune syndrome surviving respiratory failure using extracorporeal membrane oxygenation (ECMO). We also summarize thoracic insufficiency syndrome cases reported in the Extracorporeal Life Support Organization (ELSO) registry. Case Report: A two-year-old male with a chest circumference less than a third percentile for age was admitted with influenza pneumonia developing a peak oxygenation index of 103.5. The child survived to baseline pulmonary function after nine days of venous-arterial ECMO support. Discussion: The ELSO registry contained 27 individuals with a surrogate diagnosis of thoracic insufficiency (0.05%). There was no significant difference in survival to discharge for thoracic insufficiency patients (52%) compared to a previously healthy population supported with ECMO. Conclusion: ECMO is safe and may be effective in supporting individuals with thoracic insufficiency. [ABSTRACT FROM AUTHOR]

Achalasia cardia is an oesophageal motility disorder characterised by aperistalsis and failure of relaxation of the lower oesophageal sphincter. The management is predominantly palliative with focus on addressing the sphincter that involves either pneumatic dilatation or Heller myotomy which relieves dysphagia in the majority of the cases. End-stage achalasia (ESA) is characterised by failed myotomy, massively dilated and tortuous oesophagus with nutritional deterioration due to progressive dysphagia and vomiting. In these subgroups of patients, oesophagectomy may be the last resort. While oesophagectomy has been described for ESA before, thoracoscopic oesophagectomy has not been reported previously. Hereby, we report our experience of performing minimally invasive oesophagectomy (thoracoscopic) with the gastric pull-up. [ABSTRACT FROM AUTHOR]

A 46-year-old man was referred to our department due to chronic chest pain. A computed tomography showed an exophytic image arising from 5th rib that was projected on the middle lobule. The patient underwent an exploratory videothoracoscopic because we couldn't discard lung compromise. A corneal-like lesion emerging from the inner side of the 5th rib was revealed. Complete video-assited resection was done. Histopathology examination revealed a desmoplastic fibroblastoma. To our knowledge this is the first case of desmoplastic fibroblastoma arising from a rib and the second case published in all literature of chest wall involvement. [ABSTRACT FROM AUTHOR]

Ischiospinal dysostosis (ISD) is a polytopic dysostosis characterized by ischial hypoplasia, multiple segmental anomalies of the cervicothoracic spine, hypoplasia of the lumbrosacral spine and occasionally associated with nephroblastomatosis. ISD is similar to, but milder than the lethal/semilethal condition termed diaphanospondylodysostosis (DSD), which is associated with homozygous or compound heterozygous mutations of bone morphogenetic protein-binding endothelial regulator protein (BMPER) gene. Here we report for the first time biallelic BMPER mutations in two patients with ISD, neither of whom had renal abnormalities. Our data supports and further extends the phenotypic variability of BMPER-related skeletal disorders. [ABSTRACT FROM AUTHOR]

We aimed to present radiological findings of a case of pedicle hypoplasia of lumbarized S1 vertebra accompanying with multiple vertebral and rib anomalies. Congenital aplasia or hypoplasia of spine pedicle is an uncommon anomaly. Aplastic- hypoplastic cervical, thoracal, lumbar pedicles have been reported. But sacral pedicle hypoplasia is rare anomaly. Sacral spine pedicle hypoplasia of lumbosacral transitional vertebra has not been reported previously in the radiological literature. Recognising of this anomaly can prevent misinterpretation of radiologic and clinical findings. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction Open surgical procedures in the treatment of pectus excavatum (PE) involve predetermined incisions in the parasternal cartilage and the bony ribs. For some procedures, the ribs are even dissected from the sternum for better sternal mobilization and thus better elevation of the funnel. Secure restoration of the sternocostal junction is then required, with the consequence that healing may be quite impaired. Patients may also subsequently suffer from sternocostal nonunion, for example, pseudarthrosis, and dislocated ribs, as well as pain and a recurrence of PE. Materials and Methods Patients underwent another open surgery with revision of the pseudarthrotic sternocostal junctions and sufficient mobilization of the anterior chest wall, followed by an open reduction and internal fixation using Matrix Rib titanium plates (Synthes, Oberdorf, Switzerland). This procedure consisted of elevating the anterior chest wall and fixing the ribs to the sternum. In 2011 and 2012, we studied this procedure, known as elastic stable chest repair (ESCR), in a series of 20 patients. The patients underwent clinical and ultrasound examinations and X-ray radiographs after the operation, after 6 weeks, and at 3- and 12-month intervals. Results Follow-up showed high patient tolerance, although a loose plate was observed in one patient and a broken plate in three patients. A stable union was achieved for all sternocostal pseudarthroses. PE improved highly significantly (p < 0.001), as the Haller index decreased from 3.6 (range: 2.7-6.6, standard deviation [SD]: 0.92) to 2.7 (range: 2.0-3.7, SD: 0.42). Pain in the anterior chest wall was significantly reduced after the operation in the majority of cases. All but one patient was mobilized already the day after the operation. Conclusions ESCR in recurrent PE achieved functional stabilization of the anterior chest wall combined with satisfactory results. [ABSTRACT FROM AUTHOR]

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 mmvs. 302.9 ± 15.3 mmvs. 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. [ABSTRACT FROM AUTHOR]

Purpose: The application of vertebral body derotation (DVBD) is still controversial by now; the purpose of this prospective cohort study was to compare comprehensive outcomes between segmental DVBD and simple rod derotation (SRD) especially in main thoracic adolescent idiopathic scoliosis. Methods: 36 patients in DVBD group and 45 patients in SRD group were with a 2-year follow-up. Among them, 19 DVBD patients and 16 SRD patients received CT scan examinations. Results: There were no significant difference between the groups in preoperative main thoracic Cobb, apical vertebral rotation and rib hump. Apical vertebral rotation measured from CT scans was 9.7° ± 2.0° versus 15.3° ± 2.4° ( p < 0.001) postoperatively in the DVBD and SRD patients, respectively. At 2-year follow-up, the main thoracic Cobb was 14.2° ± 1.6° versus 14.7° ± 1.7° ( p = 0.18), rib hump was 6.4° ± 3.8° versus 6.8° ± 3.1° ( p = 0.60) in DVBD group and SRD group. Patients' assessments of both groups were improved in Spinal Appearance Questionnaire (SAQ) and Scoliosis Research Society-22 Questionnaire (SRS-22), but showed no significant difference at follow-up ( p = 0.47 and 0.60). Conclusion: Although segmental DVBD showed excellent radiographic correction of axial spinal deformity postoperatively, there was no more correction of clinical rib hump or better patients' assessment than SRD at follow-up in our data. [ABSTRACT FROM AUTHOR]

Background: [InlineMediaObject not available: see fulltext.]Correcting the chest wall deformity is an important goal of scoliosis surgery. A prominent rib hump deformity may not be adequately addressed by scoliosis correction alone. It has been shown that costoplasty in conjugation with scoliosis correction and instrumented spinal fusion is superior to spinal fusion alone in addressing the chest wall deformity. In cases of severe rib hump deformity unilateral convex side costoplasty alone might not adequately restore thoracic cage symmetry necessitating for additional concave side rib cage reconstruction. Case report: A 16-year-old male with adolescent idiopathic scoliosis and a sharp, cosmetically unacceptable, prominent rib hump (razorback deformity) underwent scoliosis correction with posterior spinal fusion and bilateral costoplasty. The convex-sided ribs were resected and used for concave-sided rib reconstruction. The rib hump height was reduced from 70 mm before the procedure to 10 mm after the procedure and the apical trunk rotation was reduced from 36° to 5°, respectively. Solid spinal fusion and ribs union was achieved. The patient remained very satisfied with no loss of correction at 2-year postoperative follow-up. Conclusion: Bilateral costoplasty in conjugation with scoliosis correction may provide a safe and effective method for the treatment of severe rib cage deformities associated with thoracic scoliosis. It should be considered in the presence of prominent rib hump deformity, where scoliosis correction alone or with unilateral costoplasty is unlikely to provide adequate correction. [ABSTRACT FROM AUTHOR]

ABSTRACT This study aims at assessing the mechanical behavior of a composite metal/polymer bar to be implanted in the retrosternal position, in order to correct chest wall deformities, such as Pectus Excavatum. A 300-mm-long, 12.7-mm-wide, and 3.5-mm-thick Nuss bar was considered, made of different metals and biodegradable polymers, fixed at its extremities, and with a constant force of 250 N applied on its center. Two different geometries for the metal elements to be embedded in the polymeric matrix were tested: in the former, thin metal sheets and in the latter, cylindrical metal reinforcing rods were considered. Finite element method simulation results are reported, in terms of maximum stress and strain of the bar. Furthermore, the maximum stress values obtained by varying metal sheet thickness or rod diameter (and therefore the volumetric percentage of metal within the matrix) for different material combinations are also shown; optimal configuration for the Pectus Excavatum treatment was finally identified for a composite Nuss bar. Copyright © 2014 John Wiley & Sons, Ltd. [ABSTRACT FROM AUTHOR]

A 8-year-old girl with severe pectus excavatum and an atrial septal defect had simultaneous repair of the both defects, using thransthoracic occlusion for atrial septal defect and improved Nuss technique for the pectus excavatum. Neither cardiopulmonary bypass nor sternotomy was required in this procedure. Details of the procedure and outcome are described. [ABSTRACT FROM AUTHOR]

Description of a case report of Gorlin-Goltz Syndrome diagnosed in a male newborn who presented increased head circumference and bifid ribs. Mother and grandmother presented typical physical findings of the syndrome, including palmar pits, odontogenic cysts, and history of multiple skin cancer resections. The diagnosis was based on clinical findings of three relatives. A literature review is also presented. [ABSTRACT FROM AUTHOR]

Background: VEPTR (vertical expandable prosthetic titanium rib) expansion thoracoplasty is used to manage thoracic insufficiency syndrome in early-onset scoliosis. Literature regarding the effects of this technique on pulmonary function is scarce. The aim of this study was to report the intermediate-term results of VEPTR expansion thoracoplasty.Methods: Twenty-one children with thoracic insufficiency syndrome underwent VEPTR expansion thoracoplasty from 2002 to 2012 and had complete chart data, preoperative and follow-up radiographs, and pulmonary function tests performed at the index implantation, first expansion, and last expansion. Pulmonary function tests with forced and passive deflation techniques developed for children under general anesthesia were performed prior to the index implantation and each expansion surgery under the same anesthetic conditions. Pulmonary and radiographic parameters were analyzed longitudinally.Results: Mean follow-up was six years, and mean age at implantation was 4.8 years. The mean number of expansion procedures per patient was eleven, and the mean number of pulmonary function tests was ten. The mean interval between surgical procedures was 6.4 months. Mean forced vital capacity (FVC) increased from 0.65 to 0.96 L (p < 0.0001). However, the percentage of the predicted FVC decreased from 77% to 58%. Respiratory system compliance normalized on the basis of body weight, Crs/kg, decreased by 39%, from 1.4 to 0.86 mL/cm H2O/kg. The mean Cobb angle before treatment was 80°, and the mean maximum thoracic kyphosis angle was 57° (range, 7° to 107°). The initial coronal correction was maintained at the time of final follow-up (67°); however, there was a trend toward a decrease in the maximum thoracic kyphosis angle (to 66°, p = 0.08). Clinically apparent proximal thoracic kyphosis occurred in four patients, and spinal imbalance occurred in seven. The mean gain in T1-T12 height during the treatment period was 18 mm (2.9 mm/year).Conclusions: FVC improved over time; however, this increase in lung volume did not keep up with the growth of the child, as the percentage of the predicted FVC decreased, and the chest wall stiffness increased. Coronal correction was maintained, but the increase in proximal thoracic kyphosis is concerning.Level Of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Introduction Thoracic epidurals ( TE) have been advocated as a superior method for controlling postoperative pain after repair of pectus excavatum with a Nuss procedure. However, three recent reports of permanent neurologic injury after the Nuss procedure with concurrent TE analgesia have raised concerns about the safety of this combination. Paravertebral nerve blocks ( PVNB) are used successfully for analgesia of the chest, but no studies are available comparing TE and PVNB catheters for postoperative analgesia in this patient population. This study was conducted to compare the efficacy of PVNB catheters with TE catheters for postoperative analgesia in pediatric patients undergoing the Nuss procedure. Methods We retrospectively reviewed the medical records of 20 adolescent males undergoing a thoracoscopic Nuss procedure with either bilateral PVNB catheters ( n = 10) or TE catheter ( n = 10) and compared postoperative opiate consumption and pain scores. Results There were no statistically significant differences between the groups with respect to demographics, opiate consumption measured in morphine equivalents, and pain scores. Discussion In this small series, bilateral PVNB catheters resulted in equivalent opioid consumption and pain scores when compared to TE for postoperative pain management in pediatric patients undergoing the Nuss procedure. Large prospective studies are needed to further compare the efficacy, incidence of side effects, and complications of TE and PVNB catheters for postoperative analgesia in this pediatric population. [ABSTRACT FROM AUTHOR]

Gorlin-Goltz syndrome (also known as nevoid basal cell carcinoma syndrome) was first reported in 1894, but described by Gorlin and Goltz in 1960 as a distinct entity consisting of ectodermal and mesodermal abnormalities. It is an hereditary autosomal dominant disease with a prevalence estimated in various studies to be between 1/57 000 and 1/256 000, and a male:female ratio of 1:1. We describe in brief the important radiological features of an accidentally detected case of Gorlin syndrome in the form of a pictorial interlude.

Cerebro-costo-mandibular syndrome (CCMS) describes a triad of mandibular hypoplasia, brain dysfunction and posterior rib defects ('rib gaps'). We present the CT imaging for a 2-year-old girl with CCMS that highlights the rib gap defects and shows absent transverse processes with abnormal fusion of the ribs directly to the vertebral bodies. We argue that this is likely to relate to abnormal lateral sclerotome development in embryology, with the failure of normal costo-vertebral junctions compounding impaired thoracic function. The case also highlights the use of CT for specific indications in skeletal dysplasia. [ABSTRACT FROM AUTHOR]

Objective: The Nuss procedure has become the first choice for repairing the pectus excavatum because of the advantages of the technique including minimal invasiveness and short operative duration. Although this technique appears simple and easy, lifethreatening complications during dissection such as intraoperative cardiac perforation have been reported. We developed a new approach for safer dissection of retrosternal space. Methods: We use a dissector that is commonly used for laparoscopic operation, instead of the Nuss introducer. The dissector goes through the same skin incision where the Nuss bar will be inserted. The major difference is the position of dissector insertion, which is set up more dorsally than usual, and the use of a laparoscopic dissector instead of the Nuss introducer. In this new approach, the direction of dissection is from dorsal toward the anterior thoracic wall, which allows us to visually follow the tip of the dissector throughout the surgery. Moreover using the dissector that has better manipulation capability enables us the fine dissection and also is able to precisely determine both the layer and the area of the dissection level. Results: We have treated more than 150 patients using this technique without any complications since 2008. In all the cases, safer dissection of the retrosternal space was performed with good results. Conclusion: We believe every surgeon can easily apply this procedure to patients with pectus excavatum, and this procedure can reduce the stress during the dissection. [ABSTRACT FROM AUTHOR]

Copyright of Scientia Medica is the property of EDIPUCRS - Editora Universitaria da PUCRS and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The purpose of this study was to report on the prevalence of swimming puppy syndrome (SPS) and investigate predisposing factors. Data were recorded from 2,443 puppies (1,183 males and 1,260 females) in Thailand, October 2006-September 2012, including breed, sex, number of puppies per litter, type of nest floor, number of affected limbs, and occurrence of pectus excavatum. Fifty-two puppies (2.13%) were diagnosed with SPS. The breed most frequently affected was English Bulldog (8.33%). There was no significant difference (P > 0.05) between presence and absence of disease based on sex, breed, and nest floor type. The number of puppies per litter was associated with SPS; puppies from smaller litters (1.92 ± 1.12) had a higher prevalence of the disease (P < 0.01) than puppies from larger litters (3.64 ± 2.24). Moreover, 15.38% of puppies with affected limbs showed signs of pectus excavatum (8/52); this clinical sign was more prevalent (P < 0.01) in puppies with all four limbs affected with SPS. [ABSTRACT FROM AUTHOR]

The article presents information on the study related to study the effect of isolated percutaneous thoracoplasty procedure on curve pattern in mature adolescent idiopathic scoliosis (AIS) patients. It mentions that thoracoplasty combined with spinal fusion is a commonly used procedure in scoliosis. It concludes that isolated percutaneous thoracoplasty procedure gives significant correction in rib humps with satisfactory clinical outcome.

Intrathoracic rib (IR) is a very rare anomaly in which a normal, an accessory, or a bifid rib lies within the chest cavity and may originate from a vertebra or a rib. It is more commonly present on the right side, and sometimes it may be associated with vertebral anomalies. Only 50 cases have been reported to date in the literature. In most cases, the IR is an isolated finding; it is incidentally detected and is asymptomatic. The IR can be easily missed on a chest radiograph and can be mistaken initially for a pleural lesion, lung consolidation, other peripheral lung parenchymal lesions, or a bony lesion. It is, therefore, essential for physicians and radiologists to know about this entity and consider it in the differential diagnosis, to avoid further evaluation and unnecessary investigations. We present a unique case of three intrathoracic ribs, a left second supernumerary rib, left third depressed normonumerary rib, and bifid arm of the left third rib, with block vertebrae and hypoplastic left lung. To our knowledge, this is the first such case presentation in the published literature. [ABSTRACT FROM AUTHOR]

Background: Bifid rib is usually asymptomatic but sometimes occurs associated with other pathological conditions. The aim of this study was to investigate clinical and radiological characteristics in children with bifid rib. Methods: Nine children with the diagnosis of bifid rib between 2005 and 2010 were reviewed. Chest radiography and computed tomography (CT) were performed in all patients, and 3-D images were additionally reconstructed in six patients. Results: Five girls and four boys with a mean age at presentation of 4.2 years had various types of unilateral bifid rib. Seven patients complained of a chest wall mass, whereas two patients whose costal abnormalities were incidentally detected on chest radiography were asymptomatic. Bifid ribs were confirmed on plain radiographs in six patients, while the other three patients were finally diagnosed on reconstruction 3D-CT. One patient with a flared rib on a radiograph showed bifurcation of the costal cartilage on 3D-CT. The other two patients with upper rib abnormalities on radiography had downward extension of the cervical or first rib articulating with the upper branch of the bifid first or second rib, respectively. Conclusion: Reconstruction 3D-CT can demonstrate complicated thoracic abnormalities in patients with atypical appearance of the rib on plain radiographs. An isolated bifid rib may require no further intervention. [ABSTRACT FROM AUTHOR]

Summary: Rib malformation and anatomical variations are not well known and are still often underdiagnosed. Usually, rib malformations are fortuitously discovered. We describe here the case of a girl, 4years and 4months old, who presented at the emergency unit for fever and an anterior tumefaction of the ribcage, without any other symptoms. She was eupneic with a normal pulmonary auscultation and viral tonsillitis with a negative streptococcus test. The thoracic tumefaction was parasternal, painless, and fixed and measured approximately 2.5×2cm. Ultrasound findings consisted of a duplicated and hypoechogenic hypertrophy of the sterno-costal cartilage of the 4th left rib. Magnetic resonance imaging (MRI) confirmed the diagnosis of chondral bifidity of the sterno-costal junction of the 4th left rib. Fever, due to the viral tonsillitis, disappeared after 4days. Rib malformations are rare, often anterior, unilateral, and preferentially located on the 3rd or the 4th rib. The main malformative rib lesions are bifid ribs, rib spurs, and widened ribs. Very rarely, they can be associated with Gorlin-Goltz syndrome or with other malformations such as VATER complex. The main differential diagnoses of these rib malformations are traumatic, tumoral, and infectious etiologies. In case of tumoral diseases, the topography of the lesion focuses the etiologic diagnosis: whereas an anterior and cartilaginous lesion is always benign, a lateral or posterior lesion can be an Ewing sarcoma. Rib malformation investigation consists in meticulous questioning, a complete clinical examination looking for any associated anomaly, completed by basic imaging explorations such as plain thoracic radiography focused on the ribcage and ultrasound. Finally, complementary computerized tomography or preferably MRI, depending on the anatomic location of the lesion, confirms the final diagnosis, as presented in our case report, and removes any uncertainty. [Copyright &y& Elsevier]

3-D assessment of scoliotic deformities relies on an accurate 3-D reconstruction of bone structures from biplanar X-rays, which requires a precise detection and matching of anatomical structures in both views. In this paper, we propose a novel semiautomated technique for detecting complete scoliotic rib borders from PA-0° and PA-20° chest radiographs, by using an edge-following approach with multiple-path branching and oriented filtering. Edge-following processes are initiated from user starting points along upper and lower rib edges and the final rib border is obtained by finding the most parallel pair among detected edges. The method is based on a perceptual analysis leading to the assumption that no matter how bent a scoliotic rib is, it will always present relatively parallel upper and lower edges. The proposed method was tested on 44 chest radiographs of scoliotic patients and was validated by comparing pixels from all detected rib borders against their reference locations taken from the associated manually delineated rib borders. The overall 2-D detection accuracy was 2.64 ± 1.21 pixels. Comparing this accuracy level to reported results in the literature shows that the proposed method is very well suited for precisely detecting borders of scoliotic ribs from PA-0° and PA-20° chest radiographs. [ABSTRACT FROM PUBLISHER]