Pectus

The prevalence and type of life-threatening complications related to the minimally invasive repair of pectus excavatum (MIRPE) and bar removal are unknown and underreported. The purpose of this communication is to make surgeons aware of the risk of these life threatening complications as well as the modifications which have been developed to prevent them. METHODS: Data related to life-threatening complications of Pectus Excavatum (PE) patients was obtained from four sources: 1. A survey of Chest Wall International Group (CWIG) surgeons who specialize in repairing congenital chest wall malformations, 2...

Although minimally invasive repair of pectus excavatum has been shown to have a low complication rate in large series, several case reports have documented life-threatening complications, including bleeding and cardiac perforation. We present a rare case of an arteriovenous malformation from the internal thoracic artery to the pulmonary artery caused by occlusion of the internal thoracic artery by the Nuss bar followed by an unidentified angiogenic process. The patient became symptomatic and required transcatheter coil embolization...

OBJECTIVE: To describe the autopsy case of a patient with a homozygous 2-base deletion, c171_172delGA (p.N58fs), in the C12orf65 gene. METHODS: We described the clinical history, neuroimaging data, neuropathology, and genetic analysis of the patients with C12orf65 mutations. RESULTS: The patient was a Japanese woman with a history of delayed psychomotor development, primary amenorrhea, and gait disturbance in her 20s. She was hospitalized because of respiratory failure at the age of 60...

Noonan syndrome (NS) is an autosomal dominant disorder characterized by distinctive facial features, short neck, short stature, congenital heart defects, pectus deformities, and variable developmental delays. NS is genetically heterogeneous as pathogenic variants in several genes involved in the Ras/mitogen-activated protein kinase pathway have been associated with a NS phenotype. Overall, 50% of patients harbor pathogenic variants in PTPN11, whereas 3-17% of patients have variants in RAF1. We present two premature neonates with progressive biventricular hypertrophy found to have RAF1 variants in the CR2 domain...

RATIONALE: Pulmonary nontuberculous mycobacterial (PNTM) disease often affects white postmenopausal women, with a tall and lean body habitus and higher rates of scoliosis, pectus excavatum, mitral valve prolapse, and mutations in the CFTR gene. These clinical features and the familial clustering of the disease suggest an underlying genetic mechanism. OBJECTIVES: To map the genes associated with PNTM, whole-exome sequencing (WES) was conducted in 12 PNTM families and 57 sporadic cases recruited at the NIH Clinical Center during 2001-2013...

AIM: To describe a new finding in patients with pectus excavatum - left lower lobe anterior basal segment hyperinflation [LABSH]. A secondary objective is to determine the frequency of the new finding and association with pectus excavatum severity. MATERIAL AND METHODS: The study population included 52 children who underwent preoperative computed tomography (CT) for the evaluation of pectus excavatum. A control group of 50 children was obtained after evaluating 137 CT examinations performed for other reasons...

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Minimal access procedures have revolutionized the field of surgery and opened newer challenges for the anesthesiologists. Pectus carinatum or pigeon chest is an uncommon chest wall deformity characterized by a protruding breast bone (sternum) and ribs caused by an overgrowth of the costal cartilages. It can cause a multitude of problems, including severe pain from an intercostal neuropathy, respiratory dysfunction, and psychologic issues from the cosmetic disfigurement. Pulmonary function indices, namely, forced expiratory volume over 1 s, forced vital capacity, vital capacity, and total lung capacity are markedly compromised in pectus excavatum...

AIM: In this study we analysed the early and late results of surgical treatment of asymmetrical pectus excavatum using complex surgery combining the Ravitch procedure and the Nuss procedure in the same general anaesthesia. MATERIAL AND METHODS: Eighty out of 938 patients with pectus excavatum operated on between 2002 and 2013, 67 males and 13 females aged 11 to 49 years (mean: 19.2), underwent a complex surgical procedure. During surgery the Nuss procedure was usually performed first (one corrective bar was implanted in 35 patients and two bars were inserted in 45 patients)...

AIM: To perform the prospective non-randomized single-center trial of pectus excavatum correction in adults. MATERIAL AND METHODS: For the period March 2012 - March 2016 64 patients aged 18-44 years were treated. Median age was 23 years. Male/female ratio was 41/23. All patients were included into standard survey protocol: chest MDCT, MRI of thoracic spine, pulmonary function test, echocardiography. Quality of life and complications rate after the Nuss procedure (n=50, 78...

BACKGROUND: Mutations in the COL2A1 gene cause type II collagenopathies characterized by skeletal dysplasia with a wide spectrum of phenotypic severity. Most COL2A1 mutations located in the triple-helical region, and the glycine to bulky amino acid substitutions (e.g., glycine to serine) in the Gly-X-Y repeat were identified frequently. However, the same COL2A1 mutations are associated with different phenotypes and the genotype-phenotype relationship is still poorly understood. Therefore, the studies of more patients about the recurrent mutations in COL2A1 will be needed for further research to provide more comprehensive clinical and genetic data...

BACKGROUND: Cardiac arrest is a lethal complication of Nuss repair of pectus excavatum which is strongly related to heart or big vessels injury. A rare case developed cardiac arrest without direct cardiac injury during Nuss procedure is presented in this article. CASE PRESENTATION: In July 2015, a previously healthy 18-year-old man undergoing Nuss repair for pectus excavatum developed cardiac arrest while the Nuss bar was being inserted into the chest. After successful resuscitation and exclusion of direct cardiac injury, the Nuss procedure was continued...

In children presenting with a pectus excavatum (PE) or pectus carinatum (PC) an underlying syndrome including Marfan's syndrome needs to be excluded. In adolescents, severe chest wall deformities may cause cardiac or respiratory problems but most commonly they have a psychological impact. The conservative treatment is a Vacuum Bell for PE, and a Dynamic Compression System for PC ; they play an increasing role in young patients. These devices need to be worn multiple hours per day for several months for an optimal result...

PURPOSE: To evaluate the dosimetric contribution of helical tomotherapy for breast cancers compared with conformal radiotherapy in mono-isocentric technique. PATIENTS AND METHOD: For 23 patients, the dosimetric results in mono-isocentric 3D conformational radiotherapy did not satisfy the constraints either of target volumes nor organs at risk. A prospective dosimetric comparison between mono-isocentric 3D conformational radiotherapy and helical tomotherapy was therefore carried out...

OBJECTIVE: The present study aims to elucidate the frequency of thoracic outlet syndrome after the Nuss procedure for pectus excavatum and the conditions in which thoracic outlet syndrome is likely to develop. METHODS: A retrospective study including 85 pectus excavatum patients (58 males and 27 females) was conducted. Thoracic outlet syndrome was defined as a condition in which the patient has numbness, lassitude, or pain of the upper limbs at rest or during motion of the upper limbs...

Pectus excavatum (PEXT) consists of an overgrowth of the chondral region with posterior displacement of the inferior part of the sternum, resulting in a concave chest deformity. Characteristic clinical and imaging findings may occur, depending on the compression that right cardiac chambers suffer, when squeezed between the sternum and the column vertebrae.

Pectus excavatum (PE) is the most common congenital chest wall deformity, but little is known about the influence of the Nuss surgical procedure on mental health of patients with PE. In this study, we aimed to evaluate the influence of the PE Nuss surgical procedure on mental health in Chinese patients and identify the predictors of psychological status for PE. Patients with PE (n = 266) underwent a standard surgical procedure by the same surgeon and did the Symptom Checklist 90 (SCL-90) and the Self-rating Depression Scale (SDS) questionnaires before and 1 year after surgery...

PURPOSE: The aim of our study was to evaluate thoracic vertebrae rotation in patients with pectus excavatum. Moreover, we wanted to assess the prevalence, the severity and relationship between pectus excavatum and adolescent idiopathic scoliosis (AIS). METHODS: We performed retrospective analysis of 82 preoperative chest CT in children with pectus excavatum performed between January 2008 and December 2011. For each patient Haller Index and Cobb angle was measured...

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 Nuss procedure is a recently developed technique for correction of pectus excavatum. A 23-year-old female patient presented at our emergency department with clinical signs of cardiac tamponade, which required an emergency procedure. Sixteen months ago, she underwent the Nuss procedure with a single bar. Her preoperative Haller index was 5. We report on a case of delayed recurrent cardiac tamponade that occurred 16 and 18 months after the patient underwent the Nuss procedure; in this case, we treated the patient with pericardiocentesis the first time and performed pericardial window creation with bar removal the second time...