Lower Rib Research Articles

Isolated bilateral first rib fracture associated with congenital cervical block vertebra – A case report

Fracture ribs, more frequently the lower ribs, are commonly associated with Chest trauma.1 First rib fracture is uncommon due to its peculiar anatomical location.2 It was first described by Lane (1885). Bilateral fractures of the first ribs are still rarer.2 They are usually associated with severe blunt trauma; although other mechanisms like indirect trauma, sudden contraction of the neck muscles, and stress or fatigue fractures are also been attributed.2–4 In indirect trauma, first rib fractures due to violent contraction of scalene and sternomastoid muscles.4,5 The articular processes of the neural arch have been implicated in weight transmission through the cervical spine. The exact relationship of the cervical block vertebra to the fracture ribs is not clear-cut on account of the alteration in the biomechanics. First rib fracture may be associated with early or late complications, though they are very rare.3,6,7 Treatment for isolated first rib fractures is mild analgesia and rest.2 If complications are present, each must be treated accordingly.6 To the best of our knowledge, bilateral fracture of the first rib associated with congenital cervical block vertebra has not been reported previously.

Computed Tomography Measurement of Rib Cage Morphometry in Emphysema

BackgroundFactors determining the shape of the human rib cage are not completely understood. We aimed to quantify the contribution of anthropometric and COPD-related changes to rib cage variability in adult cigarette smokers.MethodsRib cage diameters and areas (calculated from the inner surface of the rib cage) in 816 smokers with or without COPD, were evaluated at three anatomical levels using computed tomography (CT). CTs were analyzed with software, which allows quantification of total emphysema (emphysema%). The relationship between rib cage measurements and anthropometric factors, lung function indices, and %emphysema were tested using linear regression models.ResultsA model that included gender, age, BMI, emphysema%, forced expiratory volume in one second (FEV1)%, and forced vital capacity (FVC)% fit best with the rib cage measurements (R2 = 64% for the rib cage area variation at the lower anatomical level). Gender had the biggest impact on rib cage diameter and area (105.3 cm2; 95% CI: 111.7 to 98.8 for male lower area). Emphysema% was responsible for an increase in size of upper and middle CT areas (up to 5.4 cm2; 95% CI: 3.0 to 7.8 for an emphysema increase of 5%). Lower rib cage areas decreased as FVC% decreased (5.1 cm2; 95% CI: 2.5 to 7.6 for 10 percentage points of FVC variation).ConclusionsThis study demonstrates that simple CT measurements can predict rib cage morphometric variability and also highlight relationships between rib cage morphometry and emphysema.

Upregulation of skeletal muscle inflammatory genes links inflammation with insulin resistance in women with the metabolic syndrome

The metabolic syndrome, a combination of interrelated metabolic risk factors, is associated with insulin resistance and promotes the development of cardiovascular diseases and type 2 diabetes mellitus. There is a close link between inflammation and metabolic disease, but the responsible mechanisms remain elusive. The aim of this study was to identify differentially expressed genes in insulin-resistant skeletal muscle tissue of women with the metabolic syndrome compared with healthy control women. Women with the metabolic syndrome (n = 19) and healthy control women (n = 20) were extensively phenotyped, insulin sensitivity was measured using a hyperinsulinaemic euglycaemic clamp, and a skeletal muscle biopsy was obtained. Gene expression levels were compared between the two groups by microarrays. The upregulated genes in skeletal muscle of the women with the metabolic syndrome were primarily enriched for inflammatory response-associated genes. The three most significantly upregulated of this group, interleukin 6 receptor (IL6R), histone deacetylase 9 (HDAC9) and CD97 molecule (CD97), were significantly correlated with insulin resistance. Taken together, these findings suggest an important role for a number of inflammatory-related genes in the development of skeletal muscle insulin resistance.

Effects of the insertional and appositional forces of the canine diaphragm on the lower ribs.

The diaphragm has an inspiratory action on the lower ribs, and current conventional wisdom maintains that this action is the result of two mechanisms, namely, the force applied by the muscle fibres on the ribs into which they insert (insertional force) and the transmission of abdominal pressure through the zone of apposition (appositional force). The magnitude of the diaphragmatic force and the relative contributions of the insertional and appositional components, however, are unknown. To assess these forces, the inspiratory intercostal muscles in all interspaces were severed in anaesthetized dogs, so that the diaphragm was the only muscle active during inspiration, and the displacements of the lower ribs along the craniocaudal and laterolateral axes were measured during quiet breathing, during occluded breaths and during passive lung inflation. From these data, the isolated effects of pleural pressure and transdiaphragmatic pressure on rib displacement were determined. Then external forces were applied to the ribs in the cranial and the lateral direction to simulate, respectively, the effects of the insertional and appositional forces, and the rib trajectories for these external forces were used as the basis for a vector analysis to obtain the relative magnitudes of the insertional and appositional contributions to the rib displacement driven by transdiaphragmatic pressure. The results show that, per unit pressure, the inspiratory effect of the diaphragmatic force on the lower ribs is equal to the expiratory effect of pleural pressure, and that the insertional force contributes 60% of that inspiratory effect.

Measurement of Waist Circumference

OBJECTIVEWaist circumference (WC) is used to define central obesity. This study aimed to compare the performance of two recommended locations of WC measurement.RESEARCH DESIGN AND METHODSA cohort of 1,898 subjects who were without diabetes from 2006 to 2012 were followed for a median of 31 months (Taiwan Lifestyle Study). The WC-IC, recommended by the National Cholesterol Education Program Third Adult Treatment Panel, was measured at the superior border of the iliac crest, and the WC-mid, recommended by World Health Organization and International Diabetes Federation, was measured midway between the lowest ribs and the iliac crest. The abdominal subcutaneous fat area (SFA) and visceral fat area (VFA) were assessed by computed tomography.RESULTSThere was greater difference between WC-IC and WC-mid measurements in women than in men (P < 0.001). Both WC-IC and WC-mid correlated significantly with BMI, VFA, and SFA (all P < 0.001). WC-mid was better correlated to VFA than WC-IC, particularly in women, and it correlated more strongly to blood pressure, plasma glucose, hemoglobin A1c, triglyceride levels, HDL cholesterol, and C-reactive protein (all P < 0.05). The association of WC-mid with hypertension, diabetes, and metabolic syndrome was slightly better than that of WC-IC (area under the receiver operator curve 0.7 vs. 0.69, 0.71 vs. 0.68, and 0.75 vs. 0.7, respectively; all age-adjusted P < 0.05). With 90 cm (male)/80 cm (female) as criteria for central obesity, WC-mid, but not WC-IC, predicted the incidence of diabetes development (age-adjusted P = 0.003).CONCLUSIONSWC-mid is a better measurement to define central obesity than WC-IC, particularly in women.

Gorham’s Disease

Gorham's disease is a rare disorder characterized by proliferation of vascular channels. The clinical presentation of Gorham's disease is variable and depends on the site of involvement. A high index of clinical suspicion is needed for accurate diagnosis, and recognition of distinctive radiologic and histopathologic features can help facilitate and expedite diagnosis. The subsequent images were obtained from a 31-year-old male patient. Nuclear scintigraphy and computed tomography identified active bone destruction in the lower thoracic and upper lumbar spine in addition to the absence of the left lower rib cage. Gorham's disease was subsequently confirmed by biopsy.

Ion beam irradiation induced fabrication of vertical coupling waveguides

Vertically coupled waveguides have been fabricated on a silicon-on-insulator platform using a combination of reactive ion etching to pattern the device layer and high-energy proton beam irradiation followed by electrochemical etching to pattern the substrate. Infra-red light can be coupled from the lower rib waveguide within the substrate into the upper waveguide within the device layer. By varying the proton energy along the lower waveguide, we have fabricated a tapered profile which is thin at the coupling region for higher efficiency and thicker towards the outer ends for easier coupling of light. A typical coupling efficiency of 26% has been achieved.

Variations of Thoracoabdominal Volumes After Lung Transplantation Measured by Opto-Electronic Plethysmography

BackgroundLung function after lung transplantation (LTx) has been widely studied. On the contrary, the thoracoabdominal volume rearrangement after LTx has yet to be investigated. MethodsPatients with cystic fibrosis and listed for double LTx at our institution were enrolled for the prospective study to explore the effects of LTx on the rearrangement of respiratory volumes in patients affected by cystic fibrosis, by utilizing the opto-electronic plethysmography (OEP), a noninvasive method to study the volume and motion of the human trunk. Rib cage and abdominal volumes were tested with OEP (OEP system, BTS, Milano, Italy). ResultsEight patients were enrolled (male-to-female ratio: 1:3; mean age 29.3 ± 7.8 years). After LTx the volume changes analyzed with OEP revealed a significant decrease of the total lung capacity (TLC) as well as the functional residual capacity and residual volume when the chest wall volume was considered. Dividing the whole respiratory volume in the three compartments showed different trends. ConclusionsWe consider OEP a particularly useful device in patients with severe respiratory disease, in that it allows a noninvasive estimate of the volume change of the chest wall. This study demonstrates a significant reduction of thoracoabdominal volumes in patients affected by cystic fibrosis treated with bilateral LTx. Abdomen and upper rib cage were congruent with the volume reduction, while the lower rib cage showed an opposite tendency.

The effect of posture on asynchronous chest wall movement in COPD

Chronic obstructive pulmonary disease (COPD) patients often show asynchronous movement of the lower rib cage during spontaneous quiet breathing and exercise. We speculated that varying body position from seated to supine would influence rib cage asynchrony by changing the configuration of the respiratory muscles. Twenty-three severe COPD patients (forced expiratory volume in 1 s = 32.5 ± 7.0% predicted) and 12 healthy age-matched controls were studied. Measurements of the phase shift between upper and lower rib cage and between upper rib cage and abdomen were performed with opto-electronic plethysmography during quiet breathing in the seated and supine position. Changes in diaphragm zone of apposition were measured by ultrasounds. Control subjects showed no compartmental asynchronous movement, whether seated or supine. In 13 COPD patients, rib cage asynchrony was noticed in the seated posture. This asynchrony disappeared in the supine posture. In COPD, upper rib cage and abdomen were synchronous when seated, but a strong asynchrony was found in supine. The relationships between changes in diaphragm zone of apposition and volume variations of chest wall compartments supported these findings. Rib cage paradox was noticed in approximately one-half of the COPD patients while seated, but was not related to impaired diaphragm motion. In the supine posture, the rib cage paradox disappeared, suggesting that, in this posture, diaphragm mechanics improves. In conclusion, changing body position induces important differences in the chest wall behavior in COPD patients.

Impact of laparoscopic surgery on thoracoabdominal mechanics and inspiratory muscular activity

ObjectiveTo evaluate the effect of laparoscopic surgery on pulmonary volume distributions and inspiratory muscles activity. Respiratory consequences associated with postoperative pain were also evaluated. MethodsThis study enrolled 20 patients without lung disease performed spirometry and chest wall kinematic analyses (i.e., chest wall, upper and lower ribcage and abdominal volumes), and measured the activity of inspiratory muscular before and 2 days after laparoscopic surgery. Pain was also assessed. ResultsAfter laparoscopy, the patients demonstrated decreased volumes in all three thoracoabdominal compartments: abdomen (ABD), upper and lower rib cage (URC and LRC, respectively) compared with the pre-operative measurements: ABD=0.38±0.20L vs. 0.55±0.25L; URC=0.45±0.18L vs. 0.55±0.21L; and LRC=0.31±0.18L vs. 0.41±0.23L; p<0.05. A reduction in the inspiratory muscular activity after surgery was also observed (sternocleidomastoid: 10.6±5.1×10−3mV vs. 12.8±6.3×10−3mV; intercostals: 16.8±12.4×10−3mV vs. 25.1±21.3×10−3mV; p<0.05). In addition, lower volumes during deep breathing were observed in patients who reported significant pain than those who did not (0.51±0.17L vs. 0.79±0.29L; p<0.05, respectively). ConclusionLaparoscopic surgery reduces chest wall ventilation and inspiratory muscular activity during deep breathing. The effects appear to depend on the patient's reported pain level.

Diagnostic difficulties in chronic obstructive pulmonary disease exacerbations: A case report

Exacerbations of COPD (chronic obstructive pulmonary disease) influence, by their frequency and severity, the life prognosis of patients with COPD. Most exacerbations are caused by respiratory infections with negative impact on patient quality of life. The impact of frequent exacerbation in COPD is manifested by the rapid decline of lung function, decreased quality of life increased airway inflamation and high mortality. Bronchial obstruction by foreign bodies meets frequently during childhood but can occur in adults under certain conditions of loss of consciousness (sleep, administration of tranquilizers, after anesthesia, intoxicated). The longstanding intrabronchial foreign body presents problems of diagnosis and treatment which are very different from those associated with the recently inhaled foreign body. We herein report a 77-year-old male, smoker (40 pack-year) presented to the clinic with severe dyspnoea, fever and cough with purulent sputum for about 6 months. Persistent symptoms require repeated hospitalizations for receiving different regimens of antibiotics, but they have not helped. Chest radiography showed extensive consolidation of the right lower lobe, intensity rib, heterogeneous and Chest CT scan showed metallic foreign body in right lower lobar bronchus with secondary pneumonic process in the lower right lobe. Emergency was performed bronchoscopy and extracted dental crown consists of three teeth and then the patient received combined antibiotic therapy for 14 days, in association with anti-inflammatory, mucolytics and bronchodilators. After 2 weeks he was overall in very good condition and all other complaints disappeared and Chest X-ray control showed full resorption of pneumonia opacity, without disabling signs. In this particular case, only minor symptoms are seen at the beginning and the aspirated foreign body was forgotten until later symptoms (inflammation, infection) were developed and developing clinically manifest. The diagnosis was delayed due to lack of radiographic view which may be aspirated foreign body embedded in granulation tissue formed around.

Thirty One Cases of Radioguided Biopsies of Sentinel Lymph Nodes Located in the Internal Mammary Chain

INTRODUCTION: The methodology of sentinel lymph node (SLN) biopsy in breast cancer has renewed the interest in harvesting lymph nodes in internal mammary chain (IMC). The presence of the disease in IMC nodes implicates in increase of the risk for distant metastases caused by secondary seeding from persistent disease. SLN biopsy in IMC is not a troublesome procedure and could modify the indication of adjuvant chemotherapy or regional radiotherapy. However, the effect of SLN biopsy in IMC on survival still is unknown. This study examines the histopathologic involvement of sentinel nodes identified by lymphatic mapping within this drainage pathway. METHOD: We performed SLN mapping in 831 patients with early infiltrating BC staged as T1-2 N0. On the day before surgery, a solution containing dextran labeled with technetium was injected around the lesion under ultrasound or stereotaxic mammography guidance. Planar scintigraphy images were obtained with a gama camara. Among the cases in whom the SLN was mapped (hot spot) in IMC (3 SLN exclusively in IMC and 28 with simultaneous SLN in axilla), in 31 the SLN was retrieved. The IMC nodes were explored through the same incision made for removal of the primary tumor. After splitting the pectoral muscle fibers, the intercostals muscles were separated from the lower rib to expose the internal mammary vessels in contact with the SLN over the parietal pleura. Never was a rib resected. RESULTS: in 4 patients (4/31 = 12. 9%) metastatic SLNs in IMC were discovered. of them, in one occasion the nodal involvement was seen only in IMC, while in the other 3, the axillary SLNs also contained metastatic disease. Upstaging of the BC was observed in 12. 9% of the patients who were submitted to SLN biopsy in IMC, causing better selection of cases who might benefit from postoperative irradiation of IMC and systemic chemotherapy. In one patient, the internal mammary artery was damaged during the pursuit of the node, but secure hemostasis was obtained without difficulty easily. There was not any case of accidental opening of the pleural cavity. CONCLUSION: SLN biopsy in IMC provide a more accurate staging of BC patients. Prospective randomized trials are needed to determine whether the adjustments in adjuvant therapy, caused by the biopsy, are associated with improvements in disease-free and overall survival.

532 Vacterl Association: a new Case with Biotinidase Deficiency and Annular Pancreas

VACTERL (V - Vertebral anomalies, A - Anal atresia, C - Cardiovascular anomalies, T - Tracheoesophageal fistula, E - Esophageal atresia, R - Renal (Kidney) and defects, anal atresia, cardiac...

Laser beam measurement of abdominal sagittal diameter in obese children: a validation study

Sagittal diameter (SAD) has been reported to correlate to visceral fat and cardiovascular risk factors. SAD is measured with the individual lying down, halfway between the lower rib margin and the iliac crest; it represents the mid-height of the abdomen. The aim of this study was to validate SAD measured using a recently-developed laser beam device (SAD(LDB) ) against SAD measured using MRI (SAD(MRI)). Of 48 obese children (25 boys, 23 girls) aged 9-11 years on the waiting list for obesity treatment, 34 agreed to a baseline measurement, which was followed by repeated measurements 6 and 12 months later in 31 and 22 children respectively. MRI was used to examine SAD(MRI) at 5 cm above (SAD(MRI,cra) ) and below (SAD(MRI,cau)) the mid plane of the L4-5 intervertebral disc. Each of the differences SAD(LBD) - SAD(MRI, cau) and SAD(LBD) - SAD(MRI,cra) was subjected to a repeated-measurements ANOVA; the visit did not have a statistically significant effect in either case (p = 0.19 and p = 0.72, respectively). The difference SAD(LBD) - SAD(MRI, cau) was 1.50 on average (p < 0.0001; CI 1.26-1.74) while the corresponding figure for SAD(LBD) -SAD(MRI, cra) was 1.26 (p < 0.0001; CI 1.04-1.49). Regression of the difference on the mean gave slopes of -0.09 (p = 0.25) and -0.04 (p = 0.57) respectively. Prediction of SAD(MRI) from SAD(LDB) can be performed in different ways: by means of linear regression or by means of an additive correction. Thus, this laser device can be used instead of MRI to estimate SAD by using a simple correction.

Sternalis Muscle: An Uncommon Finding During Aesthetic Breast Surgery

The sternalis muscle is an uncommon anatomic variant of the chest wall musculature. It was first reported in Anatomes Elenchus Accuratissimus in 1604.1,2 Few reports of this muscle have appeared in the literature, and there is debate about its anatomic origin, insertions, function, and innervations. Reported as an inconstant finding in Netter’s Atlas ,3 the sternalis muscle is described as an analogous muscle that develops along with the rectus abdominis muscle but resorbs when chest development occurs during intrauterine life. Over the years, anatomists have coined a number of terms to refer to the sternalis muscle, such as the accessorius ad rectum and the parasternal, pectoris rectus , or praesternalis muscle .4 The sternalis is an accessory muscle that can originate from the upper sternum and the infraclavicular region, which inserts upon the anterior pectoral fascia, the lower ribs, the costal cartilages, the sheath of the rectus abdominis muscle, and the aponeurosis of the abdominal external oblique muscle. An adipose tissue layer can be interposed between the pectoralis major and sternalis muscles. Some anatomists consider the sternalis a rudiment of the panniculus carnosus in humans.5 Existence of the sternalis muscle varies between the sexes and among ethnic groups; it is reported in …

Clinical Analysis for the Correlation of Intra-abdominal Organ Injury in the Patients with Rib Fracture

BackgroundAlthough it is rare for the fracture itself to become a life threatening injury in patients suffering from rib fracture, the lives of these patients are occasionally threatened by other associated injuries. Especially, early discovery of patients with rib fracture and intra-abdominal organ injury is extremely important to the prognosis. This study analyzed the link between rib fracture and intra-abdominal injury to achieve improved treatment.Materials and MethodsAmong trauma patients that had visited the hospital emergency room from January 2007 to December 2009, a retrospective study was conducted on 453 patients suffering from rib fracture due to blunt trauma. Rib fracture was classified according to location (left, right, and bilateral), and according to level (upper rib fracture [1-2nd rib], middle rib fracture [3-8th rib], and lower rib fracture [9-12th rib]). The researched data was statistically compared and analyzed to investigate the correlation between the location, level, and number of rib fracture and intra-abdominal organ injury.ResultsMotor vehicle injury was found to be the most common mechanism of injury with 208 cases (46%). Associated injuries accompanied with rib fracture were generated in 276 cases (61%). Intra-abdominal organ injury was discovered in 97 cases (21%). Liver injury was the most common intra-abdominal injury associated with rib fracture with 39 cases (40%), followed by spleen injury, with 23 cases (23%). Intra-abdominal injury according to level of rib fracture was presented as upper rib fracture in 11 cases (11%), middle rib fracture in 31 cases (32%), and lower rib fracture in 55 cases (57%), thus verifying that intra-abdominal injuries were commonly accompanied in lower rib fractures (p=0.03). In particular, significant increase of intra-abdominal injury was presented in fractures below the 8th rib (p=0.03). The number of intra-abdominal injuries requiring emergency operations was significantly higher in patients with more than 6 rib fractures (p=0.04).ConclusionIntra-abdominal organ injury is more common in patients with lower rib fracture, especially fractures below the 8th rib. Intra-abdominal organ injuries generated in multiple rib fracture patients with more than 6 fractures significantly higher severity. These cases must be thoroughly inspected and carefully observed as there is possibility of emergency operation.

TO EXAMINE THE CLINICAL CHARACTERISTICS, PARACLINICAL SIGNS OF PATIENTS WITH LIVER CANCER AND EVALUATE THE RESULTS OF TREATMENT OF LIVER CANCER BY GAMMA KNIFE

Objective: To examine the clinical characteristics, paraclinical signs of patients with liver cancer and evaluate the results of treatment of liver cancer by gamma knife. Material and Methods: 95 patients were diagnosed liver cancer that treated with Gamma Knife in Hue University Hospital from January 2011 to May 2012. Daily monitoring of patients in treatment process and after treatment for 1 month, 3 months, 6 months...The data on clinical monitoring, images are recorded. Results and discussion: (i) Most patients with a history of hepatitis B, C (66.3%), what has not been previously treated (78.9%). Common clinical symptoms are right lower ribs pain (62.1%), poor diet (47.4%), enlarged liver (41.1%), weight loss (36.8%), fatigue (30,5%). The majority of liver tumors located in the right liver (74.8%) and usually have two or more tumors (91.6%). Large tumor size mostly bigger than 6 cm (48.4%), 43.2% of patients with tumor size 3-6cm. Hepatic enzyme increased 15.9%, 22.1% Billirubin increase, accounting for 26.3% of AFP increase. (ii) Gamma treatment with focus of more than 20 at a rate 47.4%, primarily radiation time longer than 15 minutes (58.9%). Most patients reported no abnormalities in the proces of treatment (57.9%), only a few patients have a feeling of fullness, indigestion (26.3%), pain (28.4%), dizziness (3.2%). The main clinical symptoms improved after treatment. (iii) The paraclinical biochemics, after 1-6 months of treatment patients with paraclinical signs improved. Tumor size after 1-6 months of treatment accounted for 66.3% reduction, not reduced to 22.1%, increase in size accounted for only 11.6%. Conclusion: after Gamma Knife treatment from 1 to 6 months, most patients respond well and are quite good. After one month, this rate is 84.2%. After 3-6 months, this rate is 73.1%. Keywords: liver cancer, treatment, Gamma Knife Key words: liver cancer, treatment, Gamma Knife

Tumorlike Conditions of the Pleura

Tumorlike conditions of the pleura are rare, but diagnosis is facilitated by recognizing certain imaging patterns and interpreting them in the clinical context. A tumorlike condition of the pleura is any nonneoplastic lesion of the pleura itself, or within the pleural space, that resembles a tumor. An approach to diagnosis of the tumorlike conditions of the pleura is provided, and these conditions are grouped into focal or diffuse conditions, with an emphasis on specific imaging features. Focal tumorlike conditions of the pleura include pleural plaque, thoracic splenosis, thoracic endometriosis causing catamenial pneumothorax, and pseudotumor caused by pleural effusion. Thoracic splenosis should be considered in a patient who has a healed left lower rib fracture, an absent spleen, and left lower pleural nodules. Thoracic endometriosis with catamenial pneumothorax should be considered in a woman of childbearing age who presents with right scapular pain and recurrent pneumothorax occurring at or around the onset of menses. Extrapleural hematoma is a nonpleural mimic of pleural tumor and shares some imaging features with focal tumorlike conditions of the pleura, despite residing in the extrapleural space. Diffuse tumorlike conditions of the pleura include diffuse pleural thickening and rare conditions such as Erdheim-Chester disease and diffuse pulmonary lymphangiomatosis. Erdheim-Chester disease should be considered when diffuse pleural thickening occurs with a perirenal soft-tissue halo or distal femoral sclerosis. Diffuse pulmonary lymphangiomatosis should be considered when findings include diffuse pleural thickening, interlobular septal and peribronchovascular interstitial thickening, and mediastinal fat infiltration limited to the thorax and when these findings persist despite diuretic therapy.

Late survival in Ellis–van Creveld syndrome – A case report

Ellis–van Creveld syndrome (EVC) is an autosomal recessive disorder characterized by chondrodystrophy, polydactyly, ectodermal dysplasia, and cardiac anomalies. Acromelic shortening of upper and lower limbs, genu valgum, multiple frenula, deformed teeth, short ribs and narrow thorax and congenital heart diseases complete the picture. The patients with the syndrome rarely survive into adulthood. Here, we report a lady with EVC presenting for the first time in middle age.

Effects of the Nuss procedure on chest wall kinematics in adolescents with pectus excavatum

No data are available on the effects of the Nuss procedure on volumes of chest wall compartments (the upper rib cage, lower rib cage and abdomen) in adolescents with pectus excavatum. We used optoelectronic plethysmography to provide a quantitative description of chest wall kinematics before and 6months after the Nuss procedure at rest and during maximal voluntary ventilation in 13 subjects with pectus excavatum. An average 11% increase in chest wall volume was accommodated within the upper rib cage (p=0.0001) and to a lesser extent within the abdomen and lower rib cage. Tidal volumes did not significantly change during the study. The repair effect on chest wall kinematics did not correlate with the Haller index of deformity at baseline. Six months of the Nuss procedure do increase chest wall volume without affecting chest wall displacement and rib cage configuration.