Carinatum Research Articles

Bracing of pectus carinatum: A quantitative analysis

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. Twenty-one male participants (12-17years) with chondrogladiolar PC were recruited at the time of brace fitting. Participants were evaluated on three visits: fitting, one month postfitting, and two months postfitting. Differences between prescribed force and patient-applied force were evaluated. Relationships of patient-applied force and correction rate with protrusion stiffness were assessed. Majority of individuals followed for two months (75%) had a significantly different patient-applied force (p<0.05) from their prescribed force. Protrusion stiffness had a positive relationship with patient-applied force, but no relationship with correction rate. Patients did not follow their prescribed force. Magnitudes of these differences require further investigation to determine clinical significance. Patient-applied forces were influenced by protrusion stiffness, but correction rate was not. Other factors may influence these variables, such as patient compliance. Treatment Study - Level IV.

A Distributed-Deflection Sensor With a Built-In Probe for Conformal Mechanical Measurements of Costal Cartilage at Its Exterior Surface

In this paper, a distributed-deflection sensor with a built-in probe is proposed to achieve conformal mechanical measurements of costal cartilage (CC) at its exterior surface. The sensor entails a rectangular sensing-plate integrated with a $5 \times 1$ transducer array with 0.75 mm spatial resolution underneath and a built-in probe of $0.5~{\mathrm{ mm}}\times 5~{\mathrm{ mm}}\times 3~{\mathrm{ mm}}$ above. By pressing the sensor against the exterior surface of a CC tissue with a pre-defined indentation depth pattern, the sensor conforms to the curved tissue surface via the built-in probe first, and then the mechanical properties of the tissue translate to the spatially distributed deflection in the sensor and register as resistance changes by the transducer array. One human Pectus Carinatum (PC) CC sample is measured using the sensor without a built-in probe, and two human PC samples are measured using the sensor with a built-in probe. The comparison in measured results among the three samples validates the capability of the sensor with a built-in probe for conformity to a curved tissue surface in measurements. Based on the recorded relation of average sensor deflection to indentation depth of the two samples measured using the sensor with a built-in probe, the tissue instant indentation modulus and normalized relaxation amount of the two samples are derived and found to vary significantly among the anterior/posterior surfaces and superior/inferior borders at the same position of each sample.

Minimally Invasive Repair of Pectus Carinatum

The second most common deformity of the anterior chest wall, pectus carinatum, is a diverse deformity that has been largely managed using open techniques. This study reviews clinical experience with a newly designed bar for minimally invasive repair of pectus carinatum. We reviewed the records of all patients recorded in our Chest Wall Deformities Clinical Database. Between January 2006 and November 2016, minimally invasive repair of pectus carinatum was performed in 172 patients. All met the criteria of a "compression test" of 10 to 25 kg/cm2. The mean age was 17.3 years, and 22.7% had a positive family history of a congenital chest wall deformity. Symmetric and asymmetric deformities were treated. During our study period, we designed 4 different bar configurations and their related stabilizers. All patients are assessed every 3 to 6 months. After 2 to 3 years of follow-up, the bar and the stabilizers are removed. Of 172 patients, 97.1% tolerated the procedure very well. The operation was a mean length 76.6 minutes. Average blood loss was 40 mL. Mean hospital length of stay was 3.7 days. Complications included pneumothorax, wire breakdown/rib cut, wound infection, severe pain, skin hyperpigmentation, nickel allergy, and overcorrection leading to excavatum. Patients returned to routine activity in 10 to 14 days. With a mean follow-up of 29.8 months in bar removal patients, 130 of 172 (93.8%) reported excellent results. Minimally invasive repair of pectus carinatum with the technically modified fourth-generation bar and its securing system has advantages of low morbidity, short hospital stay, and excellent cosmetic results, even in asymmetric cases.

Pectus updates and special considerations in Marfan syndrome.

Congenital chest wall or pectus deformities including pectus excavatum (funnel chest) and pectus carinatum (pigeon chest) affect a significant proportion of the general population and up to 70% of patients with Marfan syndrome. Patients often experience significant morbidity and psychological distress, which can worsen with age. Here we discuss new techniques for both operative and non-operative treatment of pectus deformity, the importance of a welltimed intervention and special considerations in patients with Marfan syndrome.

A case report of death of an adolascent with pectus carinatum (pigeon breast) deformity due to lighter gas (butane

The usage of lighter gas (butane) and similar volatile substances is an important, common and possibly fatal public health problem among young people. A significant proportion of sudden deaths due to volatile substance usage has been reported to be caused by its cardiotoxic effect. Pectus Carinatum; is the deformity of the front chest wall that protrudes forward. While it is mildly prominent at birth, it usually increases with ages (11-15 years). It was aimed to share a case of a 17-year-old male with pigeon breast deformity with literature that his autopsy and toxicological examination showed cause of death as lighter gas inhalation.

Surgical treatment of pectus carinatum in children

To improve the results of surgical treatment of pectus carinatum. The work included 47 pectus carinatum patients aged 3-15 years for the period from 2000 to 2015. All patients were divided into 2 groups depending on surgical approach: control group - 23 (48.94%) children who underwent thoracoplasty by M.M. Ravitsh procedure with the use of device for pectus carinatum repair of our design; main group - 24 (51.06%) patients where our method of thoracoplasty based on changes of deformed ribs biomechanics was used. In control group good results were obtained in 15 (65.3%) patients, satisfactory - in 5 (21.7%) patients, recurrent deformation with all consequences was observed in 3 (13%) cases. Long-term results of surgical treatment by original method were followed-up in all 24 patients and good outcomes were obtained in 100% of children. 1) Intraoperative 'spring' - effect is a provoking risk factor of recurrent chest deformation. 2) The new surgical method based on breaking the ribs bodies in 'green branch' fashion provides good results and does not require external devices to hold sternal-rib complex in corrected position.

Costal cartilage changes in children with pectus excavatum and pectus carinatum

To investigate qualitative and quantitative changes of cartilage lacunae and canals in the costal cartilages in children with PE and PC compared to those with normal chests. Costal cartilages were investigated in 10 children with normal chests (a control group), in 12 children with PE, and in 12 children with PC. Tissue fragments were fixed in 10% neutral formalin and embedded in compacted paraffin. Sections were stained with hematoxylin and eosin. Slides were examined by light microscopy. Cartilage lacunae, hyper- and hypolacunar zones, and cartilage canals were morphometrically examined, followed by statistical data analysis. There was a significant decrease in the number of cartilage lacunae and in the frequency of hyperlacunar zones and an increase in that of hypolacunar zones in the PE and PC groups. There were no significant differences in these parameters between the PE and PC groups; however, there was a tendency to the smallest number of cartilage lacunae and canals in the PC group and that to the preponderance of empty lacunae in the PE group. Only the PC group showed also negative correlations between the proportions of empty lacunae and the age of children. The pathogenesis of PE and PC in children is related to the impaired trophism of costal cartilages due to the smaller number of cartilage channels containing vessels and lacunae with chondrocytes. The development of PE and PC is associated with specific costal cartilage morphological changes that suggest that PE and PC are different manifestations of the same disease, namely connective tissue dysplasia.

Subarachnoid block a safe choice for cesarean section in neurofibromatosis patient with severe kyphoscoliosis

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. The operation was successfully performed with subarachnoid block. This case highlights the anesthetic challenges and the significance of early planning anesthetic technique.

Open repair of pectus deformity in the era of minimally invasive repair

Pectus excavatum and carinatum are the most common chest wall deformities with wide spectrum of severity and morphologic diversity. Bostanci et al . described details of traditional open technique and its modifications including removal of costal cartilages, osteotomy and fixation materials, which is a good review for open technique and very informative.

Force required for correcting the deformity of pectus carinatum and related multivariate analysis

ObjectiveTo measure the force required for correcting pectus carinatum to the desired position and investigate the correlations of the required force with patients’ gender, age, deformity type, severity and body mass index (BMI). MethodsA total of 125 patients with pectus carinatum were enrolled in the study from August 2013 to August 2016. Their gender, age, deformity type, severity and BMI were recorded. A chest wall compressor was used to measure the force required for correcting the chest wall deformity. Multivariate linear regression was used for data analysis. ResultsAmong the 125 patients, 112 were males and 13 were females. Their mean age was 13.7±1.5 years old, mean Haller index was 2.1±0.2, and mean BMI was 17.4±1.8 kg/m2. Multivariate linear regression analysis showed that the desirable force for correcting chest wall deformity was not correlated with gender and deformity type, but positively correlated with age and BMI and negatively correlated with Haller index. ConclusionsThe desirable force measured for correcting chest wall deformities of patients with pectus carinatum positively correlates with age and BMI and negatively correlates with Haller index. The study provides valuable information for future improvement of implanted bar, bar fixation technique, and personalized surgery. Type of studyRetrospective study. Level of evidenceLevel 3–4.

New Methods for Imaging Evaluation of Chest Wall Deformities.

The purpose of this study is to describe the development of an external 3-dimensional (3D) scanner as a noninvasive method for imaging chest wall deformities. It allows objective assessment, reconstruction of the area of interest, and evaluation of the severity of the deformity by using external indexes. The OrtenBodyOne scanner (Orten, Lyon, France) uses depth sensors to scan the entire 3D external body surface of a patient. The depth sensors combine structured light with two classic computer vision techniques: depth from focus and depth from stereo. The data acquired are processed and analyzed using the Orten-Clinic software. To investigate the performance of the device, a preliminary prospective study (January 2015-March 2016) was carried out in patients attending our hospital chest wall deformities unit. In total, 100 patients (children and young adults) with pectus excavatum or pectus carinatum, treated by surgery or non-operative methods were included. In patients undergoing non-operative treatment, external 3D scanning was performed monthly until complete correction was achieved. In surgically treated patients, scanning was done before and after surgical correction. In 42 patients, computed tomography (CT) was additionally performed and correlations between the Haller index calculated by CT and the external Haller index using external scanning were investigated using a Student's test (r = 0.83). External scanning is an effective, objective, radiation-free means to diagnose and follow-up patients with chest wall deformities. Externally measured indexes can be used to evaluate the severity of these conditions and the treatment outcomes.

Intentional Lower Pole Rotation of Anatomic Breast Implants in Chest Wall Deformities.

Several methods have been described for the correction of congenital thoracic wall deformities. Our aim was to investigate the feasibility and clinical results of using standard anatomic breast implants with modified anatomic positioning according to the defect in congenital thoracic wall deformities. Between 2014 and 2015, 5 patients diagnosed with pectus excavatum (PE, n = 4) or pectus carinatum (PC, n = 1) and breast asymmetry or hypoplasia were evaluated. In all patients, a submammary incision and dual-plane subpectoral placement of texturized, anatomic implants were performed. In patients with PE, the lower pole of the implant was positioned medially to compensate for the caved chest. In patients with PC, the lower pole of the anatomic implant was positioned laterally to compensate for the prominent sternum. Outcome measures were satisfaction, minor and major complications, and morbidity. The mean surgery time was 95 ± 14 minutes, and the mean implant volume was 287 ± 56 cm3 (273 ± 60 cm3 on the right side and 305 ± 60 cm3 on the left side). After a median follow-up of 25 months (range: 2-35), all patients healed uneventfully, and a satisfactory correction of the thoracic wall deformity was achieved. Thus, by adjusting the lower pole of anatomic breast implants in a horizontal plane according to the thoracic defect, we showed satisfactory results. Our technique has a low complication rate and can be recommended for the correction of mild to moderate PE or PC.

Minimally Invasive Repair of Pectus Carinatum

Minimally Invasive Repair of Pectus Carinatum

The effect on cardiopulmonary function after thoracoplasty in pectus carinatum: a systematic literature review.

Creating an aesthetically appealing result using thoracoplasty, especially when correcting extensive deformities, but only causing low morbidity, is challenging. The frequency of thoracoplasties in cases of pectus carinatum (PC) has increased due to improved experience and modified surgical techniques, resulting in low morbidity and low complication rates. The indications for surgical treatment are still controversial and, in most cases, remain aesthetic or psychological rather than physiological. However, whether cardiopulmonary function changes after surgical repair remains a matter of controversy. We sought to investigate and shed light on published knowledge regarding this question. We searched MEDLINE and PubMed databases, using various defined search phrases and inclusion criteria, to identify articles on pre- and postoperative cardiopulmonary evaluation and outcomes. Six studies met the inclusion criteria: 5 studies evaluated patients with PC for cardiopulmonary outcomes after chest wall surgery and 1 did so following conservative compression treatment. In these studies, surgical and conservative correction of PC did not reduce absolute lung volumes and spirometric measurements and consequently had no pathogenic effect on cardiopulmonary function. The results of this systematic review suggest that surgical correction of PC has no symptomatic pathogenic effect on cardiopulmonary function. The results, however, revealed both heterogeneity in the examinations used and inconsistent methods within each study. Further prospective trials with a stronger methodological design are necessary to objectively confirm that surgical correction of PC does not impair cardiopulmonary function.

Bir olgu nedeni ile mukopolisakkaridoz tip 4 (Morquio sendromu)

Objective: Mucopolysaccharidoses, develop with genetic transmission which cause destructive changes in various organs as a result of glycasoaminoglycans deposition in lysosomes. Herein, we are presenting a case with Morquio syndrome so as to attract attention to this rarely seen disease. Case: A 11-year-old male patient presented to our hospital with complaints of short stature, and bone deformities. The parents were not relatives, and he had 3 healthy younger siblings. On physical examination, coarse facial features, hypertelorism, disproportionate short stature, genu valgum, pectus carinatum, kyphoscoliosis, were detected. He demonstrated a waddling gait. His neuromotor development was normal for his age. On bone radiograms, kyphoscoliosis, flared iliac wings, coxa valga, flattening of femoral epiphysis, widened ribs, and narrowed iliac wings were detected. Urine tests were positive for glycosaminoglycans (keratan sulphate). Enzymatic analysis revealed low levels of glucose-6-phosphatase [15 pmol/mg/hr, (n:400-2000)], which made the final diagnosis. Conclusions: Morquio syndrome has Aand B types with similar clinical manifestations which are transmitted as an autosomal recessive trait. Tip 4A is a disease due to a deficiency of galactosamine-6-sulfatase (GALNS) enzyme, which is encoded by the 16q24.3 gene, while Type 4B is characterized by deficiency of beta galactosidase. In both types of the disease, keratan sulphate, and chondrotin 6 sulphate accumulate. Also in our case manifestations became more prominent around age 1, and gradually progressed. Our patient without mental problems had typical skeletal signs on radiograms which is named dysostosis multiplex. It is necessary to initiate enzyme replacement therapybefore the onset of gait disorders.

A Single-Center Experience with Dynamic Compression Bracing for Children with Pectus Carinatum.

Bracing for pectus carinatum (PC) has emerged as an alternative to surgical correction. However, predictive factors for bracing remain poorly understood, as much of the data have been reported from small series. We reviewed a prospective dataset in patients with PC who underwent dynamic compression bracing (DCB) from July 2011 to July 2016. Bracing was initiated in patients > 10 years of age with a significant PC and desire for bracing. Data were analyzed for those observed two or more times after the brace was fitted to the patient. A total of 503 patients were evaluated for PC and 340 (68%) underwent DCB. Eighty-five percent were males with an average age of 14 ± 2 years. There was a positive correlation of age with pressure of initial correction (PIC, r = 0.2). One patient underwent operative correction as the initial therapy. Two hundred seventeen patients had two or more visits after the patient was fitted for the brace. The mean PIC in this cohort was 4 psi (range: 1.5-7.8), and the median duration of bracing in this group was 16 months (IQR: 7-23 months). One hundred three patients (47%) achieved complete correction after an average bracing time of 7.5 months and were then placed in the retainer mode. Thirty patients successfully completed bracing therapy and required an average of 23 months of therapy (2 months-4 years). No patient recurred after bracing was completed, but one failed bracing and required operative correction. Complications included mechanical problems (8%), skin complications (10%), complaints of tightness (3%), and pain (2%). DCB has both early and lasting effects in the correction of PC with minimal complications. Predictive factors for successful resolution of the PC include increased duration of DCB and lower initial PIC.

Pain and sensory disturbances following surgical repair of pectus carinatum.

The purpose of this study was to assess the characteristics of persistent postoperative pain and sensory disturbances following surgical repair of pectus carinatum. Using a prospective observational design, 28 patients were assessed before, 6 weeks and 6 months after a modified Ravitch operation for pectus carinatum. Postoperative pain was assessed using the Short Form McGill Pain Questionnaire. Sensory testing was conducted to detect brush-evoked allodynia and pinprick hyperalgesia. Additionally, generic and disease-specific quality of life was assessed using the Short Form-36 Health Survey and the Nuss Questionnaire Modified for Adults before and after surgery. Six weeks after surgery, ten patients reported mild pain or discomfort. Six months after surgery, four patients reported only mild pain. Allodynia was detected in two patients 6 weeks and 6 months after surgery. Hyperalgesia was detected in eight patients 6 weeks after surgery, and in six patients 6 months after surgery. Generic quality of life was significantly improved over time. The study showed no significant pain problems, a tendency to reduced sensory disturbances and significant improvements in quality of life 6 months after surgical repair of pectus carinatum. Future studies should include a longer follow-up period to determine if these positive results are persistent. 1 (Prognosis Study).

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 43years. Patients were observed for 6months to 8years (mean of approximately 3years). 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 1cm 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 .

Recurrent c.G1636A (p.G546S) mutation of COL2A1 in a Chinese family with skeletal dysplasia and different metaphyseal changes: a case report

BackgroundMutations 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. In this paper, we report a rare recurrent c.G1636A (p.G546S) mutation in COL2A1 associated with different metaphyseal changes in a Chinese family.Case presentationThe proband (III-3) was the second child of the family with skeletal dysplasia. She was 2 years and 3 months old with disproportional short stature, short neck, pectus carinatum, genu varum, bilateral pes planus, and obvious waddling gait. Notably, she displayed severe metaphyseal lesions, especially typical “dappling” and “corner fracture” appearance, whereas no particular metaphyseal involvement was detected in the proband’s mother (II-3) and elder sister (III-2) in the family. We identified a heterozygous mutation (c.1636G > A) in COL2A1 in the three patients, causing the substitution of glycine to serine in codon 546. Although the same mutation has been reported in two previous studies, the phenotypes of the previous patients were different from those of our patients, and the characteristic “dappling” and “corner fracture” metaphyseal abnormalities were not reported previously.ConclusionsIn this study, we identified a c.G1636A (p.G546S) mutation in the COL2A1 associated with different metaphyseal changes, which was never reported in the literature. Our findings revealed a different causative amino acid substitution (glycine to serine) associated with the “dappling” and “corner fracture” metaphyseal abnormalities, and may provide a useful reference for evaluating the phenotypic spectrum and variability of type II collagenopathies.

Модифікація методу Мартінеза–Ферро у лікуванні кільоподібної деформації грудної клітки у дітей

Модифікація методу Мартінеза–Ферро у лікуванні кільоподібної деформації грудної клітки у дітей