Evaluating the web as a source of information for patients with chest wall deformities: insights into engagement and disparities.

BACKGROUND: Recent data highlight the internet's pivotal role as the primary information source for patients. In this study, we emulate a patient's/caregiver's quest for online information concerning chest deformities and assess the quality of available information. METHODS: We conducted an internet search using combination of the terms "pectus excavatum," "pectus excavatum surgery," "funnel chest," "pectus excavatum repair" and identified the first 100 relevant websites from the three most popular search engines: Google, Yahoo, and Bing. These websites were evaluated using the modified Ensuring Quality Information for Patients (EQIP) instrument. RESULTS: Of the 300 websites generated, 140 (46.7%) were included in our evaluation after elimination of duplicates, non-English websites, and those targeting medical professionals. The EQIP scores in the final sample ranged from 8 to 32/36, with a median score of 22. Most of the evaluated websites (32.8%) originated from hospitals, yet none met all 36 EQIP criteria. DISCUSSION: None of the evaluated websites pertaining to pectus excavatum achieved a flawless "content quality" score. The diverse array of websites potentially complicates patients' efforts to navigate toward high-quality resources. Barriers in accessing high-quality online patient information may contribute to disparities in referral, patient engagement, treatment satisfaction, and overall quality of life. LEVEL OF EVIDENCE: IV.

Spinal posture, mobility, and position sense in adolescents with chest wall deformities: a comparison of pectus excavatum, pectus carinatum and healthy peers.

PURPOSE: The study aimed to compare spinal posture, mobility, and position sense in adolescents with pectus excavatum (PE), pectus carinatum (PC), and healthy control (HC). METHODS: 22 with PE, 22 with PC, and 21 HC were included in the study. The spinal posture (thoracic kyphosis, lumbar lordosis, pelvic tilt, thoracic, lumbar, pelvic lateral tilt angles) and mobility (thoracic, lumbar, hip/sacral, and overall, in the sagittal and frontal plane) with the spinal mouse, and spinal position sense (repositing errors) with the inclinometer were assessed. RESULTS: The thoracic kyphosis angle of PE and PC was higher than in HC (p < 0.001; p = 0.001). Hip/sacral mobility in the sagittal plane was lower in the PE and PC than control, respectively (p < 0.001; p < 0.001). Overall sagittal spinal mobility (p:0.007) and hip/sacral mobility in the frontal plane (p:0.002) were lower in the PC than in HC. Overall frontal spinal mobility was lower in the PE and PC than in HC (p:0.002; p:0.014). The PE and PC repositing errors were higher (p < 0.001; p:0.014). CONCLUSION: The study found that adolescents with PE and PC had decreased spinal mobility, spinal alignment disorders, and a decline in spinal position sense. It is important not to overlook the spine during physical examinations of adolescents with chest wall deformities. In clinical practice, we suggest that adolescents with chest deformities should undergo a spine evaluation and be referred for physical therapy to manage spinal disorders.

Postoperative Complications of Flap Procedures in Chest Wall Defect Reconstruction: A Two-Center Experience.

Background and Objectives: Chest wall defect reconstruction is a complex procedure aimed at restoring thoracic structural integrity after trauma, tumor removal, or congenital issues. In this study, postoperative complications were investigated to improve the care of patients with these critical conditions. Materials and Methods: A retrospective study of chest wall reconstructions from 2004 to 2023 was conducted at Klinikum Nürnberg and Evangelisches Waldkrankenhaus Spandau-Berlin. Data included patient demographics, comorbidities, defect etiology, surgery details, and complications using the Clavien-Dindo classification. Results: Among the 30 patients included in the study, a total of 35 complications occurred in 35 thoracic wall defect reconstructions. These complications were classified into 22 major and 13 minor cases. Major complications were more common in patients with cancer-related defects, and considerable variations were observed between free flap and pedicled flap surgeries. Notably, the use of the anterolateral thigh (ALT) flap with vastus lateralis muscle demonstrated promise, exhibiting fewer complications in select cases. The reconstruction of chest wall defects is associated with substantial complications regardless of the etiology of the defect and the particular surgical procedure used. Interestingly, there was a lower complication rate with free flap surgery than with pedicled flaps. Conclusions: The ALT flap with vastus lateralis muscle deserves further research in this field of reconstruction. Multidisciplinary approaches and informed patient discussions are crucial in this complex surgical field, emphasizing the need for ongoing research and technique refinement.

Three-Dimensional Imaging of the Chest Wall: A Comparison Between Three Different Imaging Systems.

BACKGROUND: Three-dimensional (3D) imaging is being used progressively to create models of patients with anterior chest wall deformities. Resulting models are used for clinical decision-making, surgical planning, and analysis. However, given the broad range of 3D imaging systems available and the fact that planning and analysis techniques are often only validated for a single system, it is important to analyze potential intrasystem and intersystem differences. The objective of this study was to investigate the accuracy and reproducibility of three commercially available 3D imaging systems that are used to obtain images of the anterior chest wall. METHODS: Among 15 healthy volunteers, 3D images of the anterior chest wall were acquired twice per imaging device. Reproducibility was determined by comparison of consecutive images acquired per device while the true accuracy was calculated by comparison of 3D image derived and calipered anthropometric measurements. A maximum difference of 1.00 mm. was considered clinically acceptable. RESULTS: All devices demonstrated statistically comparable (P = 0.21) reproducibility with a mean absolute difference of 0.59 mm. (SD: 1.05), 0.54 mm. (SD: 2.08), and 0.48 mm. (SD: 0.60) for the 3dMD, EinScan Pro 2X Plus, and Artec Leo, respectively. The true accuracy was, respectively, 0.89 mm. (SD: 0.66), 1.27 mm. (SD: 0.94), and 0.81 mm. (SD: 0.71) for the 3dMD, EinScan, and Artec device and did not statistically differ (P = 0.085). CONCLUSIONS: Three-dimensional imaging of the anterior chest wall utilizing the 3dMD and Artec Leo is feasible with comparable reproducibility and accuracy, whereas the EinScan Pro 2X Plus is reproducible but not clinically accurate.

Physician-Estimated Depth as a Screening Tool for Computed Tomography Evaluation of Pectus Excavatum.

BACKGROUND: Pectus excavatum (PE) is the most common congenital chest wall anomaly with a reported incidence of 1/300 to 1/400 live births and a male predominance. Preoperative evaluation of defect severity typically requires a calculation of the Haller index (HI) and/or correction index (CI) using computed tomography (CT) or x-rays. The purpose of this study was to determine whether physician-estimated depth (PED), a bedside screening tool, could be used to identify a subset of pediatric patients in whom CT was unnecessary. METHODS: After institutional review board approval (IRB #032018-091), we retrospectively reviewed all patients with a diagnosis of PE between 2009 and 2018 at our academic pediatric center. Demographic information including age, sex, and body mass index were abstracted. Imaging was reviewed to obtain HI and CI and to retrospectively calculate PED. The PED is calculated at the bedside by measuring the depth of the pectus at the site of greatest depression relative to a horizontal surface laid across the deformity. For this retrospective study, we calculated the CT-derived PED by measuring the depth from the horizontal on the respective CT images. Patients without imaging studies and patients with pectus carinatum, arcuatum, or mixed deformities were excluded from this study. RESULTS: A total of 94 patients met inclusion criteria. Of these, 82% were male, with a median age of 15 y. Patients were further subdivided by BMI, with 46% of patients having a BMI of <18.5 kg/m2 (i.e., underweight), whereas 54% of patients had a BMI of ≥18.5 kg/m2. Using a threshold PED of 2 cm, patients with a BMI of <18.5 kg/m2 had correct classification rates of 93% and 95% using PED relative to HI and CI, respectively. Patients with a BMI of ≥18.5 kg/m2 had correct classification rates of 80% and 88% using PED relative to HI and CI, respectively, at the same 2 cm threshold. CONCLUSIONS: PED is a viable screening tool for the preoperative evaluation of PE with a 2 cm threshold providing the combination of high sensitivity, specificity, and correct classification rates especially in underweight patients.

A new ovine model of spine and chest wall deformity at birth with alteration of respiratory system mechanics and lung development: a feasibility study.

PURPOSE: To develop an animal model of spine and chest wall deformity (CWD) at birth and to evaluate its effects on respiratory system mechanics and lung development. METHODS: A spine and CWD was created in utero between 70 and 75 days of gestation in six ovine fetuses by resection of the seventh and eighth left ribs. Two days after birth, respiratory system mechanics was assessed in anesthetized lambs using the flexiVent apparatus, followed by postmortem measurement of lung mechanics as well as histological lung analysis. RESULTS: A range of severity of CWD was found (Cobb angle from 0° to 48°) with a mean decrease in compliance of 47% and in inspiratory capacity of 39% compared to control lambs. Proof-of-concept histological analysis in one lamb showed marked lung hypoplasia. CONCLUSION: Our ovine model represents a pilot proof-of-concept study evaluating the impact of a spine and CWD present at birth on lung respiratory mechanics and development. This study lays down the groundwork for future studies evaluating the impact of these deformities on lung development and potential treatments. These slides can be retrieved under Electronic Supplementary Material.

An Evidence-Based Approach to Management of Pectus Excavatum and Carinatum.

Pectus excavatum (PE) and pectus carinatum (PC) are the most common congenital chest wall anomalies. Current research suggests that PE and PC may result from overgrowth of the sternocostal cartilages. This can produce a deformation that displaces the sternum inward as in PE or outward as in PC. The etiology, clinical presentation, evaluation, and management of PE and PC are reviewed. Varied clinical presentations, cardiopulmonary effects, and psychosocial aspects are described.

VEPTR: Are We Reducing Respiratory Assistance Requirements?

BACKGROUND: The assisted ventilation rating (AVR) indicates the degree of external respiratory support required in children with thoracic insufficiency syndrome (TIS) and early onset scoliosis. For skeletally immature patients with TIS, the vertical expandable prosthetic titanium rib (VEPTR) device can be used to improve lung volume and growth. We hypothesized that patients who underwent early thoracic reconstruction by VEPTR treatment had an improved respiratory status. METHODS: Preoperative and postoperative AVR ratings were prospectively collected in a multicenter study group and compared to determine change after VEPTR treatment. Patients under 10 years of age at initial implant with minimum of 2-year follow-up data were included. Patients were excluded if there were incomplete data or if initial AVR was normal (breathing on room air). Statistical analysis was performed on groups which had stable, declined, and improved AVR at final follow-up. RESULTS: Database search yielded 77 patients with initial abnormal AVR. Average follow-up was 5.6 years. The most frequent primary diagnoses were congenital scoliosis (n=14) and spinal muscular atrophy (n=14). In total, 19 (24%) demonstrated improvement, 9 (12%) patients deteriorated, and 49 (64%) remained at the same level. The average preoperative major curve in those with improvement (58.4 degrees) and those with no change (63.5 degrees) was less than in those with deterioration (85.5 degrees) (P=0.014). The average age in years at implant of those with improvement (4) was less than those declined (6.7) and those with no change (5.5). In total, 16 (84.2%) of those that improved had a normal AVR and did not require respiratory support at last follow-up. CONCLUSIONS: There is evidence that a subset of patients with early onset scoliosis and TIS who received early thoracic reconstruction with VEPTR treatment show complete resolution of pulmonary support at final follow-up. In total, 89% of 79 patients did not experience respiratory deterioration. A total of 24% (n=19) had a positive change with over 84% (n=16) of this group no longer requiring support. LEVEL OF EVIDENCE: Level III-prognostic.

Risk of Primary Spontaneous Pneumothorax According to Chest Configuration.

BACKGROUND: We compared the chest configurations of patients with primary spontaneous pneumothorax (PSP) and age-sex-matched controls to determine the presence of chest wall deformities in patients with PSP. METHODS: We retrospectively enrolled 166 male patients with PSP (age, 18-19 years) and 85 age-sex-matched controls without PSP, who simultaneously underwent chest computed tomography (CT) and radiography at one of two institutes. After correcting for height, the following thoracic parameters were comparatively evaluated between the two groups: maximal internal transverse (T) and anteroposterior (W) diameters of the chest, maximal internal lung height (H), Haller index (T/W), and T/Height, T/H, W/Height, W/H, and H/Height ratios. RESULTS: Patients were taller than the control subjects (176.5 cm ± 5.9 cm versus 174.4 cm ± 5.6 cm; p = 0.007). After controlling for height, the patient group exhibited lower T and W and greater H and Haller index values than the control group (T: 95% confidence interval [CI], 24.8-25.2 cm versus 25.9-26.5; W: 95% CI, 8.9-9.2 cm versus 10.1-10.6 cm; H: 95% CI, 25.2-25.9 cm versus 23.4-24.4 cm; and Haller index, 95% CI, 2.7-2.9 versus 2.4-2.6; all, p < 0.001). The patient group also exhibited lower T/Height, T/H, W/Height, and W/H ratios and greater H/Height ratio than the control group. CONCLUSIONS: Patients with PSP have an anteroposteriorly flatter, laterally narrower, and craniocaudally taller thorax than subjects without PSP, suggesting that chest configuration is associated with the development of pneumothorax.

Chest wall mass: what lies beneath?

CLINICAL INTRODUCTION: A 24-year-old Filipino man attended the ED with a 1-month history of a discrete swelling over his upper anterior chest wall that was rapidly increasing in size and tenderness. He denied any other symptoms. His medical history was unremarkable.Examination revealed a tender, 7 cm × 6 cm mass over the upper part of the sternum (figure 1). The surface was smooth, it was immobile, non-compressible and the overlying skin was normothermic but mildly erythematous. Cervical lymphadenopathy was present. His vital signs were normal.emermed;35/10/586/F1F1F1Figure 1Chest wall mass. QUESTION: What is the most likely diagnosis?LipomaChondrosarcomaLymphoma Mycobacterium tuberculosis (TB).

A confounding rib variation: bilateral symmetric aberrant posterior rib articulations and bridgings.

INTRODUCTION: Although mild bone angulation with osseous enlargement often suggests fractures with callus formation, in some cases the diagnosis is synchondrosis. CASE REPORT: We present a rare variation of the chest wall in a 15-year-old male with a history of lymphoma. Bilateral multi-level posterior rib enlargements revealing mild 18F-fluorodeoxyglucose uptake were detected via positron-emission tomography/computed tomography. The variations were identified as healing fractures, although the more accurate diagnosis was determined to be multi-level posterior rib synchondroses with consecutive bridgings. Although variant bone anatomies are commonly seen in radiological practice, such multiple symmetrical posterior rib synchondroses associated with consecutive bridgings and articulations have not been clearly demonstrated before. CONCLUSION: Awareness of such a rare combination of a well-known variation is crucial for radiologists to exclude malignancies, possibility of fracture and suspicion of child abuse.

Breast Asymmetry in Women Requesting Plastic Surgery of the Breast.

OBJECTIVE: Breast asymmetries have a high prevalence among women requesting breast augmentation. However, the prevalence of breast asymmetries in women undergoing other types of breast surgeries is not known. METHODS: The breast measurements of consecutively women evaluated for plastic surgery of the breast, but without prior breast surgery, were prospectively recorded in a plastic surgery database. They were classified into three groups according to the presenting breast problem: hypoplastic breasts, macromastia, and ptotic breasts. Comparisons were made between the right and left side of each patient regarding the symmetry of the nipple-areola complex (size and position), breast mound, and chest wall. RESULTS: The breast measurements of 304 women were analyzed. The mean age was 35 ± 12 years. The study population was distributed in the following manner: 126 hypoplastic breast cases, 100 macromastia cases, and 78 ptotic breast cases. Asymmetry of the position of the nipple-areola complex was found in 54%, 59%, and 56% of the groups, respectively. Asymmetry of the breast mound volume was found in 41%, 47%, and 44% of the groups. Asymmetry of the chest wall was present in 12%, 11%, and 10% of the groups, respectively. Overall, we found that 91% of the cases had at least one type of breast asymmetry. Prevalence of asymmetry was not different (p>0.05) among the groups, but the magnitude was larger in macromastia. CONCLUSION: Breast asymmetries were detected in the majority of women and the prevalence was similar across the different groups, however the magnitude was greater in hypertrophic breasts.

Invasive versus non-invasive ventilation for acute respiratory failure in neuromuscular disease and chest wall disorders.

BACKGROUND: Acute respiratory failure is a common life-threatening complication of acute onset neuromuscular diseases, and may exacerbate chronic hypoventilation in patients with neuromuscular disease or chest wall disorders. Standard management includes oxygen supplementation, physiotherapy, cough assistance, and, whenever needed, antibiotics and intermittent positive pressure ventilation. Non-invasive mechanical ventilation (NIV) via nasal, buccal or full-face devices has become routine practice in many centres. OBJECTIVES: The primary objective of this review was to compare the efficacy of non-invasive ventilation with invasive ventilation in improving short-term survival in acute respiratory failure in people with neuromuscular disease and chest wall disorders. The secondary objectives were to compare the effects of NIV with those of invasive mechanical ventilation on improvement in arterial blood gas after 24 hours and lung function measurements after one month, incidence of barotrauma and ventilator-associated pneumonia, duration of mechanical ventilation, length of stay in the intensive care unit and length of hospital stay. SEARCH METHODS: We searched the following databases on 11 September 2017: the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE and Embase. We also searched conference proceedings and clinical trials registries. SELECTION CRITERIA: We planned to include randomised or quasi-randomised trials with or without blinding. We planned to include trials performed in children or adults with acute onset neuromuscular diseases or chronic neuromuscular disease or chest wall disorders presenting with acute respiratory failure that compared the benefits and risks of invasive ventilation versus NIV. DATA COLLECTION AND ANALYSIS: Two review authors reviewed searches and independently selected studies for assessment. We planned to follow standard Cochrane methodology for data collection and analysis. MAIN RESULTS: We did not identify any trials eligible for inclusion in the review. AUTHORS' CONCLUSIONS: Acute respiratory failure is a life-threatening complication of acute onset neuromuscular disease and of chronic neuromuscular disease and chest wall disorders. We found no randomised trials on which to elaborate evidence-based practice for the use of non-invasive versus invasive mechanical ventilation. For researchers, there is a need to design and conduct new randomised trials to compare NIV with invasive ventilation in acute neuromuscular respiratory failure. These trials should anticipate variations in treatment responses according to disease condition (acute onset versus acute exacerbation on chronic neuromuscular diseases) and according to the presence or absence of bulbar dysfunction.

Incidence and Classification of Chest Wall Deformities in Breast Augmentation Patients.

Although chest wall deformities (CWDs) are seen four times more often in males than in females, most patients who consult plastic surgery clinics in our country are females. Breast augmentation and augmentation mastopexy were performed with a total of 812 breast implants in 406 patients. Forty-three of these patients had various CWDs. The CWD patient ages ranged from 18 to 43 years. Patients were observed for 6 months to 8 years (mean of approximately 3 years). Pectus excavatum (PE) was present in 22 patients, pectus carinatum (PC) in five patients, Poland syndrome (PS) in two patients, sunken chest deformity (SCD) in three patients, barrel chest deformity (BCD) in five patients, body builder deformity (BBD) in three patients, and long upper chest wall (LCW) in three patients. None of the patients had functional or cardiac deformities. A total of 86 round, anatomically textured gel implants in various profiles were used within a range of implant volumes for all patients. There were no serious complications in the 43 CWD patients. The frequency of CWDs in the total population of 406 patients was approximately 10.6%. PE was present in 51% (22/43) of the patients with CWDs. The deformity rates among patients who underwent augmentation mammoplasty were as follows (%): PE, 5.4; PC, 1.23; PS, 0.5; SCD, 0.73; BCD, 1.23; BBD, 0.73; and LCW, 0.73. The three most common deformities observed in this study were PE, PC, and BCD. The least common deformity was PS. Patients with CWDs should be carefully evaluated preoperatively, and all the associated measurements and calculations must be meticulously performed. The implant selection may differ according to the deformity pattern. For example, in patients with PE, prostheses with larger base diameters cover the deformity better. Although high-profile prostheses are preferred on the affected side in cases of PS and SCD, low- or middle-profile prostheses are preferred on the opposite side. Despite this common consensus, there was an asymmetry of approximately 1 cm between the sides, as observed postoperatively. However, the patients did not express dissatisfaction with this situation. Low-profile prostheses should be preferred for PC and BCD protruding CWDs because asymmetry becomes more prominent and over-projection occurs more frequently in cases of high-profile prostheses. The BBD projection is also a problem. For this reason, high-profile prostheses should be preferred in these patients. It is possible to obtain satisfactory results by using appropriate breast implants in patients with CWDs. Level of Evidence IV This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Chest Wall Constriction after the Nuss Procedure Identified from Chest Radiograph and Multislice Computed Tomography Shortly after Removal of the Bar.

BACKGROUND: This study radiographically examined the changes in the chest walls of patients with pectus excavatum (PE) after Nuss bar removal, to define the deformation caused by the bar and stabilizer. In the first part of the study, we compared the changes in chest radiographs of patients with PE to a preoperation PE control group. In the second part, we used multislice computed tomography (CT) scans to provide three-dimensional reconstructions with which to evaluate the changes to the thoracic wall. METHODS: Part 1 From June 2006 to August 2011, 1,125 patients with PE who had posteroanterior chest radiographs taken before undergoing the Nuss procedure at four hospitals were enrolled as a preoperative control group. At the same time, 203 patients who had the bar removed were enrolled as the study group. The maximum dimensions of the outer boundary of the first to ninth rib pairs (R1-R9, rib pair width), chest height, and chest width were measured. Part 2 Thirty-one consecutive patients with PE (20 males and 11 females) who underwent Nuss bar removal were evaluated 7 to 30 days after operation. During this period, a further 34 patients with PE who had undergone CT imaging before bar insertion were evaluated and compared with the postoperative group. RESULTS: Part 1 The width of the lower ribs (R4-R9) after bar removal was significantly less than in the age-matched controls. The ribs adjacent to the bar (R5-R7) showed the greatest restriction. The width of the upper ribs (R1-R3) 2 to 3 years after bar placement did not differ significantly from the controls. Patients who were operated on after 10 years of age had less of a restrictive effect. Three years of bar placement resulted in more restriction than a 2-year period, particularly in patients younger than 10 years old. Part 2: A significant constriction of the chest wall was observed in 13 patients after removal of the Nuss bar. Constriction at ribs 5 to 8 was found to be present adjacent to the site of bar insertion. However, constriction of the chest wall was found in only 3 of the 34 patients in the preoperative group. The severity of constriction (as graded by the spline model) also increased in the postoperative group. CONCLUSION: The growth of the chest wall was restricted after placement of the Nuss bar for PE correction. Long-term follow-up of chest wall growth is needed to clarify whether such constriction resolves with time.

Chest wall hypoplasia--principles and treatment.

The chest is a dynamic structure. For normal movement it relies on a coordinated movement of the multiple bones, joints and muscles of the respiratory system. While muscle weakness can have clear impact on respiration by decreasing respiratory motion, so can conditions that cause chest wall hypoplasia and produce an immobile chest wall. These conditions, such as Jarcho-Levin and Jeune syndrome, present significantly different challenges than those faced with early onset scoliosis in which chest wall mechanics and thoracic volume may be much closer to normal. Because of this difference more aggressive approaches to clinical and surgical management are necessary.

A Cross-Sectional Study of Chest Wall Development in Patients with Pectus Excavatum.

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.

Elastic Stable Chest Repair as a Means of Stabilizing the Anterior Chest Wall in Recurrent Pectus Excavatum with Sternocostal Pseudarthrosis: An Innovative Fixation Device.

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.

Thoracoschisis: A Case Report and Review of Literature.

Thoracoschisis is an extremely rare congenital birth defect in which intra-abdominal organs eviscerate through a defect in the thoracic wall(1). There are only seven previously reported pediatric cases and in each case, there is some diaphragmatic anomaly, suggesting that the defect took place before complete formation of the diaphragm. Our patient was referred to us from a local hospital immediately after delivery. The patient was born with a thoracoschisis of the left side below the 8(th) intercostal space. The thoracoschisis was repaired. Although there is a high prevalence of cardiac defects among thoracoschisis patients, this patient shows only small atrial septal defects.

Possible Genetic Origin of Limb-Body Wall Complex.

Limb body wall complex (LBWC) is characterized by multiple severe congenital malformations including an abdominal and/or thoracic wall defect covered by amnion, a short or absent umbilical cord with the placenta almost attached to the anterior fetal wall, intestinal malrotation, scoliosis, and lower extremity anomalies. There is no consensus about the etiology of LBWC and many cases with abnormal facial cleft do not meet the requirements for the true complex. We describe a series of four patients with LBWC and other malformations in an attempt to explain their etiology. There are several reports of fetuses with LBWC and absent gallbladder and one of our patients also had polysplenia. Absent gallbladder and polysplenia are associated with laterality genes including HOX, bFGF, transforming growth factor beta/activins/BMP4, WNT 1-8, and SHH. We postulate that this severe malformation may be due to abnormal genes involved in laterality and caudal development.