Minimally invasive correction of asymmetric chest deformity in children

Purpose. To analyze literature data and to find out optimal techniques for surgical correction of asymmetrical chest wall deformity in children.Material and methods. The researchers studied data on surgical treatment of children with asymmetrical chest wall deformity who had thoracoplasty performed with well-known techniques developed by Bairov, Timoshchenko, Paltia, Ravitch, Kondrashin which included the resection of crooked cartilage and internal metal fixation with consideration of deformity shape, location and severity. For comparison, the authors analyzed the data on the minimally invasive Nuss technique and its modifications.Results. The researchers found that thoracoplasty by the Bairov method is less effective which gives the largest number of postoperative complications (16.5%) in the form of hemothorax, pneumothorax and hydrothorax what significantly increased the length of hospital stay and the following rehabilitation process. On the contrary, minimally invasive interventions were the most effective, if to compare with all applied techniques for chest wall deformity correction, having a low percentage of complications; though their drawback is long-term postoperative analgesia.Conclusion. At present, there is no any consensus on the top-priority technique for surgical correction of chest wall deformity in children. It necessitates further research in this direction as well as improvement of the existing techniques and development of new ones.

PECTUS EXCAVATUM: A CASE REPORT OF AN EARLY REPAIR

Introduction: Pectus Excavatum usually recognized during infancy and become worse with growing ages. Case presentation: We presented a 6 months old male child to our departmental OPD with complaints of indrawing of anterior chest wall. On physical examination revealed a signicant Pectus Excavatum deformity. There was no other abnormality except the chest wall deformity. An abnormal chest X-ray and CT scan demonstrated leftward displacement of heart and great vessels. Conclusion: Surgery for Pectus Excavatum is specialized and can be done in early childhood with better outcome.

Lung Transplantation for Pleuroparenchymal Fibroelastosis

Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the prevalence and incidence, clinical and pathological characteristics, and disease course of PPFE. Patients with PPFE reportedly have several unique clinical characteristics—including an extremely low body mass index with a slender body and chest wall deformity, known as “flat chest”. As this disease progresses, shrinking of the lungs often causes life-threatening complications, such as pneumothorax, and associated air leak syndrome. Lung transplantation is considered the only effective treatment for patients with advanced PPFE; however, little is known about the influences of the characteristics of PPFE on the outcome of lung transplantation. This review focuses on the unique clinicopathologic characteristics of PPFE and associated outcomes of lung transplantation for these patients.

Impact of chest wall deformity on cardiac function by CMR and feature-tracking strain analysis in paediatric patients with Marfan syndrome

Objectives To evaluate systolic cardiac dysfunction in paediatric MFS patients with chest wall deformity using cardiac magnetic resonance (CMR) imaging and feature-tracking strain analysis. Methods Forty paediatric MFS patients (16 ± 3 years, range 8−22 years) and 20 age-matched healthy controls (16 ± 4 years, range 11−24 years) were evaluated retrospectively. Biventricular function and volumes were determined using cine sequences. Feature-tracking CMR was used to assess global systolic longitudinal (GLS), circumferential (GCS) and radial strain (GRS). A dedicated balanced turbo field echo sequence was used to quantify chest wall deformity by measuring the Haller index (HI). Results LV volumes and ejection fraction (EF) were similar in MFS patients and controls. There was a trend for lower right ventricular (RV) volume (75 ± 17 vs. 81 ± 10 ml/m2, p = 0.08), RV stroke volume (41 ± 12 vs. 50 ± 5 ml/m2, p < 0.001) and RVEF (55 ± 10 vs. 62 ± 6%, p < 0.01) in MFS patients. A subgroup of MFS patients had an increased HI compared to controls (4.6 ± 1.7 vs. 2.6 ± 0.3, p < 0.001). They demonstrated a reduced RVEF compared to MFS patients without chest wall deformity (50 ± 11% vs. 58 ± 8%, p = 0.01) and controls (p < 0.001). LV GLS was attenuated when HI ≥ 3.25 (- 16 ± 2 vs. - 18 ± 3%, p = 0.03), but not GCS and GRS. LV GLS (p < 0.01) and GCS (p < 0.0001) were attenuated in MFS patients compared to controls, but not GRS (p = 0.31). RV GLS was attenuated in MFS patients compared to controls (- 21 ± 3 vs. - 23 ± 3%, p < 0.05). Conclusion Chest wall deformity in paediatric MFS patients is associated with reduced RV volume, ejection fraction and GLS. Feature-tracking CMR also indicates impairment of systolic LV function in paediatric MFS patients. Key Points • Paediatric Marfan patients demonstrate reduced RV volume and ejection fraction compared to healthy controls. • A concordant attenuation in RV global longitudinal strain was observed in Marfan patients, while the RV global circumferential strain was increased, indicating a possible compensatory mechanism. • Subgroup analyses demonstrated alterations in RV ejection fraction and RV/LV global strain parameters, indicating a possible association of severe chest wall deformity with biventricular dysfunction in paediatric Marfan patients.

Extra-thoracal migration of the Nuss Bar in corrective surgery for pectus excavatum: a very rare late complication

Pectus excavatum is a chest wall deformity with an incidence of around 1 in 400 live births. The Nuss procedure is a surgical intervention that aims to restore functional integrity in these patients. We report the first case of bar migration into the stomach necessitating further surgical intervention for removal. Our case presents a rare complication of pectus excavatum repair and highlights the importance of vigilant follow-up in these patients. If bar migration does occur, imaging in the form of X-ray and CT scans may be of use in early detection in order to expedite management.

The correlation between spinal and chest wall deformities and pulmonary function in Marfan syndrome

Purpose Scoliosis, chest wall deformities and pulmonary involvement are common features of Marfan syndrome (MFS). We aimed to assess the impact of spinal and chest wall deformities on pulmonary function in paediatric MFS patients with a surgically managed spinal deformity. Methods In this multicentre retrospective study, spirometry, lung volumes and radiographic imaging were performed on 26 MFS patients between the ages of seven and 18 years who were undergoing planned spinal fusion surgery for spinal deformity. A correlation analysis assessed the relationship between radiographic measurements of spinal and chest wall deformities and predicted total lung capacity (TLC), forced vital capacity (FVC) and the ratio between forced expiratory volume in one second and FVC (FEV1/FVC). Results In total, 18 patients (70%) had impaired pulmonary function. Thoracic kyphosis (mean 19.3°; -32° to 54°) had a strong positive correlation with FEV1/FVC (r = 0.65; p < 0.001). Significant decrease in FEV1/FVC below 80% occurred at kyphosis under 15° (p = 0.004). Kyphosis had a moderate negative correlation with FVC (r = -0.43; p = 0.03). Chest wall deformity had a strong negative correlation with FEV1/FVC (r = -0.61; p = 0.001). The magnitude of the thoracic curve (mean 55.2°; 28° to 92°) had a significant moderate negative correlation with TLC (r = -0.45; p = 0.04). Conclusion In MFS, three factors correlate with decreased pulmonary function measures: hypokyphosis, increasing chest wall deformity and increasing coronal curve magnitude. Hypokyphosis and increased chest wall deformity correlated with diminished FEV1/FVC; increasing thoracic spinal curvature with diminished TLC. Further analysis with a larger cohort will help better define the relationship between these deformities and pulmonary function in this unique population. Level of Evidence IV