Pectus carinatum

Objective: To analyze the clinical characteristics of patients with Mucopolysaccharidosis ⅣA (MPS ⅣA). Methods: A retrospective study was conducted on 111 patients with MPS ⅣA in Xinhua Hospital of Shanghai Jiao Tong University School of Medcine from December 2008 to August 2020, confirmed by enzyme activity and genetic testing. General situation, clinical manifestations and enzyme activity test results were analyzed. According to the clinical manifestations, it can be divided into severe, intermediate and mild group...

OBJECTIVES: To explore the correlation between the Haller index (HI), the external depth of protrusion, and the external Haller index (EHI) for both pectus excavatum (PE) and carinatum (PC). To assess the variation of the HI during this first year of non-operative treatment for pectus deformities in children. METHODS: From January 2018 to December 2022, all children treated for PE by vacuum bell and for PC by compression therapy at our institution were evaluated by external gauge, 3D scanning (iPad with Structure Sensor and Captevia-Rodin4D), and magnetic resonance imaging (MRI)...

BACKGROUND: We aim to describe four new subtypes of PE in detail and represent modification of the Nuss procedure called crossed bar technique for their optimum correction with good results. METHODS: 101 patients who underwent crossed bar technique between August 2005 and February 2022 were included into the study. RESULTS: The mean age of the patient series was 21.1 (range 15-38 years) years. Mean Haller index was 3.87. Mean operation duration was 86...

Pectus carinatum (PC) is a chest deformity caused by disproportionate growth of the costal cartilages compared with the bony thoracic skeleton, pulling the sternum forwards and leading to its protrusion. Currently, the most common non-invasive treatment is external compressive bracing, by means of an orthosis. While this treatment is widely adopted, the correct magnitude of applied compressive forces remains unknown, leading to suboptimal results. Moreover, the current orthoses are not suitable to monitor the treatment...

3q29 deletion syndrome (3q29del) is a rare genomic disorder caused by a 1.6 Mb deletion (hg19, chr3:195725000â€"197350000). 3q29del is associated with neurodevelopmental and psychiatric phenotypes, including an astonishing >40-fold increased risk for schizophrenia, but medical phenotypes are less well-described. We used the online 3q29 registry ( 3q29deletion.org ) to recruit 57 individuals with 3q29del (56.14% male) and requested information about musculoskeletal phenotypes with a custom questionnaire...

Evaluation of the sternum is part of the routine examination of small animal thoracic radiographs. However, descriptions on frequency and type of abnormalities are lacking. This retrospective observational study aimed to describe abnormal radiographic findings of the sternum in a cross-section of client-owned dogs and cats undergoing thoracic radiography between 1 January 2019 and 1 January 2021 for reasons unrelated to diseases of the sternum. The study population consisted of 777 dogs (mean age, 7.3 ± 3...

BACKGROUND: Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers. METHODS: Multicenter retrospective study of young patients' files diagnosed with pectus arcuatum...

BACKGROUND: This study aims to investigate the prevalence of chest deformity in middle- and high-school students in Sivas province of Turkey, to identify the risk factors associated with the psychological and physical disorders caused by the deformity, and to facilitate early diagnosis and treatment guidance by increasing awareness of this problem. METHODS: Between October 2011 and May 2012, a total of 15,862 students (8,508 males, 7,354 females; mean age: 15.9±1...

BACKGROUND: This study aimed to evaluate the congenital anomalies of ribs in the Turkish population using multi-detector computed tomography (CT) and to reveal the prevalence and distribution of these anomalies according to genders and directions. MATERIALS AND METHODS: This study included 1120 individuals (592 male, 528 female) over 18 who applied to our hospital with a suspicion of Covid-19 and who had thoracic CT. Anomalies such as a bifid rib, cervical rib, fused rib, SRB anomaly, foramen rib, hypoplastic rib, absent rib, supernumerary rib, pectus carinatum, and pectus excavatum, which were previously defined in the literature, were examined...

INTRODUCTION: Mindsets of health have been linked to different outcomes for patients with both surgical and medical conditions. A "growth" mindset, in which health is defined as malleable and subject to improvement, is associated with improved attitudes and outcomes when compared to a "fixed" mindset, in which health is defined as unchangeable. In pediatric surgery, parental growth mindsets of health have been correlated with better postoperative outcomes, including lower scores of anxiety and pain perceptions, for children...

INTRODUCTION: Children's Interstitial Lung Diseases (cHILD) are a heterogeneous group of rare respiratory diseases. Their common characteristics are gas exchange abnormalities and diffuse pulmonary infiltrates on chest imaging. This group includes inherited surfactant protein deficiency (ISPD), a little-known etiology in Tunisia. CASE PRESENTATION: A 22-month-old boy was referred to investigate recurrent respiratory infections. He had polypnea, cyanosis, finger clubbing, pectus carinatum, intercostal retraction, and bilateral crackles on pulmonary auscultation...

The ADNP -gene-related neurodevelopmental disorder Helsmoortel-Van der Aa syndrome is a rare syndromic-intellectual disability-an autism spectrum disorder first described by Helsmoortel and Van der Aa in 2014. Recently, a large cohort including 78 patients and their detailed phenotypes were presented by Van Dijck et al., 2019, who reported developmental delay, speech delay and autism spectrum disorder as nearly constant findings with or without variable cardiological, gastroenterological, urogenital, endocrine and neurological manifestations...

Cardiac surgery is usually completed through the median sternotomy. If the sternum is not fixed firmly after the operation, it may lead to secondary thoracic deformities. The most common deformity after operation is pectus carinatum, and the relatively less common deformity is pectus excavatum, but the compound thoracic deformity has not been reported. We met a 3-year-old boy who underwent surgery for congenital ventricular septal defect at the age of 1. He developed a severe compound thoracic deformity after surgery...

Pectus carinatum can be treated with a single minimally invasive surgery, but the severe cases will appear secondary depression after treatment, which needs to be corrected additionally. We used the combined Wenlin procedure and Wung procedure to treat a patient with severe pectus carinatum and achieved satisfactory results.

OBJECTIVE: To evaluate the sagittal tomographic reformatting of the sternum using unpublished radiographic parameters (indexes and angles), comparing them between the different types of pectus, and controls. METHODS: 44 patients with pectus deformities and controls underwent chest CT for analysis. The types of pectus were classified into: inferior pectus carinatum (IPC), superior (SPC) and lateral (LPC), and broad (BPE) and localized pectus excavatum (LPE). The following tomographic parameters were created and measured: (1) spine-manubrium-sternum index (SMS); (2) column-sternum index (CSI); (3) manubrium-sternal angle (MSA); (4) inferior manubrium angle (IMA); and (5) inferior sternum angle (ISA)...

The TNNT1 gene encoding the slow skeletal muscle TnT has been identified as a causative gene for nemaline myopathy. TNNT1 nemaline myopathy is mainly characterized by neonatal-onset muscle weakness, pectus carinatum and respiratory insufficiency. Herein, we report on a Chinese girl with TNNT1 nemaline myopathy with mild clinical phenotypes without thoracic deformities or decreased respiratory function. Muscle biopsy showed moderate to marked type 1 fiber atrophy and nemaline rods. Next-generation sequencing identified the compound heterozygous c...

INTRODUCTION: Surgical repair of pectus excavatum and carinatum in children has historically been associated with severe postoperative pain and prolonged hospitalization. Enhanced Recovery After Surgery (ERAS) is a multidisciplinary, multimodal approach designed to fast-track surgical care. However, obstacles to implementation have led to very few within pediatric surgery. The aim of this study is to outline the process of development and implementation of an ERAS protocol for pectus surgical repair using fundamental principles of implementation science...

BACKGROUND: Severe presentation of primary hyperparathyroidism (PHPT) which, although, common in the Asian population, is albeit rarely encountered in the present era. CASE PRESENTATION: We present a 23-year-old lady whose symptoms began in 2016 with generalized body aches. Subsequently, her husband noticed a reduction in her height and she developed gradual protrusion of chest. Meanwhile, she conceived in November 2019 and delivered a full-term healthy baby girl...

BACKGROUND: Pectus carinatum is a pediatric condition that can be treated by dynamic compression system (DCS) bracing or surgery. Several publications on DCS bracing or surgery are available; however, they do not compare both treatments. METHODS: Over a 10-year period, 738 patients with pectus carinatum were treated at the Amsterdam Pectus Center (Amsterdam, The Netherlands). This study describes this 10-year experience and the results of both treatments. RESULTS: Of the 631 patients who underwent DCS bracing treatment, 553 finished treatment, and 78 patients are still under treatment...

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