Pectus

Minimally invasive procedure for the treatment of pectus excavatum as described by Nuss has been used from 1987. The bar initially introduced blindly is now introduced under thoracoscopic control to increase safety of the procedure. It is usually removed two to three years after its insertion in a one-day procedure. Complications of the bar removal are rare but potentially serious. We report the case of a serious complication which occurred immediately after the Nuss bar removal. A 15-year-old boy underwent a Nuss procedure for a severe pectus excavatum without relevant complication...

Introduction: Pectus excavatum repair (Nuss procedure) is a painful procedure requiring effective postoperative analgesia. Aim: To establish whether thoracic epidural analgesia with ropivacaine is non-inferior to epidural analgesia with bupivacaine following the Nuss procedure in children. Material and methods: The prospective, randomized, controlled, single blind study included 81 children. Computer-generated random numbers were used to allocate treatment...

PURPOSE: The Nuss procedure for surgical correction of pectus excavatum often causes severe postoperative pain. Cryoanalgesia of intercostal nerves is an alternative modality for pain control. We describe our modification of the cryoICE™ probe that allows for nerve ablation through the ipsilateral chest along with early results utilizing this technique. METHODS: To allow for ipsilateral nerve ablation, a 20-French chest tube was cut and secured to the cryoICE probe, thus providing insulation for the malleable end of the probe...

Introduction: Additional use of the video-assisted thoracoscopic surgery (VATS) technique in the Nuss procedure has been globally accepted for the improvement of safety of surgical treatment as well as for decreased frequency of serious intraoperative and postoperative complications. Aim: To evaluate VATS in surgical treatment of patients with pectus excavatum by the left-to-right Nuss procedure for prevention of serious intra- and postoperative complications. Material and methods: From 2002 to 2016, 1006 patients with pectus excavatum aged 7 to 62 years (mean: 18...

BACKGROUND AND PURPOSE: This study was designed to investigate clinical and pathologic characteristics of five Korean patients with RYR1-related congenital myopathy (CM). METHODS: Five patients from unrelated families were diagnosed with RYR1-related CM via direct or targeted sequencing of RYR1. Their clinical, mutational, and pathologic findings were then analyzed. RESULTS: Seven different mutations were identified, including two novel mutations: c...

Neurofibromatosis type 1 (NF1) is an autosomal-dominant neuroectodermal hereditary disorder, in which spinal skeletal deformities are one of the manifestations. Pectus carinatum, temporomandibular joint dysfunction, and kyphoscoliosis can be seen with this genetic disorder which can lead to cardiorespiratory system problems and can cause difficulty in airway management. We present the anesthetic management of a NF1 patient with a sharp thoracolumbar dystrophic kyphoscoliosis who have been posted for emergency cesarean section as a result of fetal distress...

Mucopolysaccharidoses (MPS) are a group of lysosomal storage disorders that affect regulation of glycosaminoglycan (GAG) processing. In MPS, the lysosomes cannot efficiently break down GAGs, and the specific GAGs accumulated depend on the type of MPS. The level of impairment of breakdown varies between patients, making this one of the many factors that lead to a range of clinical presentations even in the same type of MPS. These clinical presentations usually involve skeletal dysplasia, in which the most common feature is bone growth impairment and successive short stature...

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BACKGROUND/PURPOSE: The Nuss procedure to correct pectus excavatum is associated with severe postoperative pain. The purpose of this retrospective study was to compare pain management outcomes of thoracic epidural analgesia and continuous infusion of local anesthetic (CILA) with and without preoperative self-hypnosis training (SHT) after Nuss procedure (4 treatment groups). METHODS: Between February 2010 and December 2013, 24 of 53 adolescents who underwent Nuss procedure received SHT...

The incidence of pectus excavatum has been estimated to be between 0.1% and 0.8% though a large autopsy series reports. After publication of the Nuss procedure for pectus excavatum, it became widely accepted. However, there are still some complications, such as over-correction and recurrence. To reduce differences in the procedure due to surgeons' experience level, preoperative simulation may be useful. Thus, we performed simulated surgery using a specific patient's three-dimensional (3D) chest wall model made by a 3D printer before operation...

PURPOSE: The purpose of this study was to determine variables predictive of an excellent correction using vacuum bell therapy for nonoperative treatment of pectus excavatum. METHODS: A single institution, retrospective evaluation (IRB 15-01-WC-0024) of variables associated with an excellent outcome in pectus excavatum patients treated with vacuum bell therapy was performed. An excellent correction was defined as a chest wall depth equal to the mean depth of a reference group of 30 male children without pectus excavatum...

BACKGROUND: The minimally invasive repair of pectus excavatum (MIRPE) has been widely accepted and has become a viable alternative to the open Ravitch technique. MIRPE has evolved over time with some advocating that a safe repair can be accomplished without direct visualization utilizing thoracoscopy. The MIRPE with and without a thoracoscopic approach has not been previously analyzed from a nationwide database to determine differences in safety and short-term outcomes. METHODS: The American College of Surgeons National Surgical Quality Improvement Program-Pediatric (NSQIP-P) 2012-2015 database was used in identifying patients that had MIRPE using Current Procedural Terminology (CPT) codes and ICD-9CM/ICD-10CM postoperative diagnosis codes...

Background: Supernumerary marker chromosomes derived from chromosome 5 (SMC5) and 5p13 duplication syndrome are rare disorders, and phenotypic descriptions of patients are necessary to better define genotype-phenotype correlations for accurate, comprehensive genetic counseling. The purpose of this study is to highlight the unique findings of a patient with a 5p13.3-q11.2 duplication arising from a SMC5 and compare and contrast the phenotype with cases in the literature. Case presentation: We report on an adult male with a 22 Mb duplication of chromosome 5p13...

The aim of the study was to correlate the clinical severity of pectus excavatum with its type and degree based on objective radiographic evaluation. Twelve brachycephalic dogs were included. Grading of the clinical severity was done based on a 6-point grading score. Thoracic radiographs were used to calculate the frontosagittal and vertebral indices at the tenth thoracic vertebra and the vertebra overlying the excavatum. Correlation between the clinical severity score and frontosagittal and vertebral indices was evaluated using Pearson's correlation coefficient...

Sterile sternotomy nonunion is a recognized complication after median sternotomy. It is defined as sternotomy that persists after 3 months without evidence of bony healing but with healing of the overlying soft tissues. It is a morbid condition associated with pain and sternal instability. We present two challenging cases of sterile sternotomy nonunion after cardiac operations that were treated successfully with novel methods adopted from the Elastic Stable Chest Repair for complex pectus deformity repair, using transverse costal to costal external cortical plates and bicortical screws, after debridement, autologous bone grafting and double loop wire sternal approximation...

BACKGROUND: Pectus excavatum, the most common congenital chest wall deformity in pediatric patients, leads to pulmonary dysfunction. There is no consensus regarding the effectiveness of the Nuss procedure for recovering pulmonary function. In this meta- analysis, we focused on the changes that occur in pulmonary function after the Nuss procedure. METHODS: We performed a literature search in the MEDLINE, Embase, Cochrane library and PubMed databases. The included studies were required to contain pulmonary function tests with results adjusted to predicted values both before and after the Nuss procedure...

BACKGROUND: Current approaches to quantifying the severity of pectus excavatum (PE) require internal measurements based on cross-sectional imaging. The aim of this study is to exploit a novel index evaluated on the external surface of the chest with a 3D optical scanner. METHODS: 51 children (41 males, 10 females), between 2 and 17 years old, were evaluated with a 3D optical scanner. PE severity was calculated by using an ad hoc instant 3D scanner and defining an automatic procedure to generate an optical 3D index (CI-3D)...

Pectus excavatum is the most common congenital deformity of the chest. The Nuss procedure is minimally invasive surgical correction of this defect, using retrosternal metal bars. The purpose of the present study was to describe a 15-year experience with the Nuss surgery, and to evaluate the long-term clinical results of the procedure. We retrospectively evaluated 239 patients, aged 14-34, who underwent the Nuss surgery in the years 2002-2016. Postoperative complications were observed in 40/236 (16.9%) patients...

BACKGROUND/PURPOSE: Primary treatment of pectus carinatum (PC) is performed with an external brace that compresses the protrusion. Patients are 'prescribed' a brace tightening force. However, no visual guides exist to display this force magnitude. The purpose of this study was to determine the repeatability of patients in applying their prescribed force over time and to determine whether the protrusion stiffness influences the patient-applied forces and the protrusion correction rate...

BACKGROUND: Carbohydrate sulfotransferase 11 (CHST11) is a membrane protein of Golgi that catalyses the transfer of sulfate to position 4 of the N-acetylgalactosamine residues of chondroitin. Chondroitin sulfate is the predominant proteoglycan in cartilage, and its sulfation is important in the developing growth plate of cartilage. A homozygous deletion encompassing part of the gene and the embedded miRNA MIR3922 had been detected in a woman with hand/foot malformation and malignant lymphoproliferative disease...