Carinatum Research Articles

Evaluating Inter- and Intraobserver Agreement on Pectus Carinatum Severity and Treatment Outcomes: A Comparison of Subjective and Objective Assessment Methods.

Background Visual examination is crucial for assessing pectus carinatum (PC) severity and treatment results. This cross-sectional study evaluates the inter- and intraobserver agreement of PC deformities before and after treatment. Methods Observers examined medical photographs of patients before and after treatment. Primary outcome was inter- and intraobserver agreement on esthetic results after treatment. Secondary outcomes included inter- and intraobserver agreement on severity and symmetry before treatment, differences in esthetic results after Ravitch surgery and dynamic compression bracing (DCS-bracing), and the impact of scars, age and treatment duration on esthetic results. Results Medical photographs of 201 patients (aged 4-18) were evaluated by five surgeons and five peers. Surgeons and peers demonstrated inadequate (κ<.61) interobserver agreement on esthetic results (κ=.26, κ=.22), severity of PC (κ=.43, κ=.38) and symmetry (κ=.37, surgeons only). Agreement between surgeons and peers on esthetic results (κ=.37) and severity before treatment (κ=.54) was similarly inadequate. Surgeons and peers demonstrated inadequate intraobserver agreement on esthetic results (κ=.49, κ=.34), severity of PC (κ=.54, κ=.48) and symmetry (κ=.60, surgeons only). Deformities treated with Ravitch surgery were perceived as more severe but yielded better results. Peers, unlike surgeons, viewed scars as negatively impacting results. No relationship was found between results after treatment and treatment duration (p=.682, p=.062) or age ( p=.205, p=.527). Conclusions Subjective assessment of PC severity and esthetic results is inconsistent. 3D-scanning could help standardize treatment completion and aid patients and surgeons in determining treatment completion. The psychosocial effects of scars should be addressed when discussing treatment options.

Exploring chest wall deformities in childhood and adolescence: insights from a case-control study

BackgroundChest wall deformities, though relatively uncommon in childhood and adolescence, significantly affect aesthetic perception and can impact cardiac and respiratory function. This study aims to compare individuals with pectus deformities to a healthy control group, shedding light on the condition’s etiology and prognosis.MethodConducted as a case-control study between 2020 and 2022, the research included a case group of 71 patients with pectus excavatum or pectus carinatum who were followed up at the Pediatric Cardiology outpatient clinic. The control group consisted of 80 children without chronic diseases. Researchers retrospectively reviewed patient files, recording demographic data, echocardiography findings, and serum levels of calcium, phosphate, vitamin D, and alkaline phosphatase.ResultsPatients with chest wall deformities exhibited significantly lower serum levels of vitamin D, calcium, and phosphorus compared to those without deformities. Males constituted 77% of the case group, with 15.5% exhibiting abnormal echocardiogram results, with mitral valve anomalies being most prevalent.ConclusionWhile pectus deformities may lead to cardiac or respiratory issues, patient concerns often center around aesthetics. Unlike typical studies focused on surgical techniques and postoperative patients, our study focused on all diagnosed patients. Findings emphasize the importance of monitoring calcium, phosphorus, and vitamin D levels in pectus patients to manage surgical risks and facilitate recovery.

Safety and efficacy of high thoracic epidural analgesia for chest wall surgery in young adolescents: A retrospective cohort analysis and a new standardised definition for success rate.

Chest wall surgery for the correction of pectus excavatum or pectus carinatum has gained increased interest in recent years. Adequate pain treatment, respiratory physiotherapy and early ambulation are key to improving the outcomes. Although thoracic epidural analgesia is highly effective, its safety is controversial, leading to extensive scrutiny and questioning of its role. We hypothesise that thoracic epidural analgesia is effective and well tolerated to use in adolescents, with a high success rate and low pain scores. Observational retrospective cohort study. All adolescent cases in a high-volume academic tertiary chest wall surgery centre between March 1993 and December 2017 were included. A total of 1117 patients aged from 12 to 19 years of age and receiving either Ravvitch, Nuss or Abramson chest wall reconstruction for pectus excavatum were identified in our institutional chest wall surgery database. After applying selection and exclusion criteria, 532 patients were included in the current analysis. The primary endpoint of this study was the safety of epidural analgesia, assessed by the incidence of acute adverse events. Secondary endpoints were block success rates using a specific novel definition, and analgesic efficacy using recorded postoperative pain scores. More than 60% of patients experienced one or more adverse events. However, all events were minor and without consequences. No serious or long-term adverse events were detected. The success rate of thoracic epidural placement was 81%. Low postoperative pain scores were observed. Thoracic epidural analgesia is an extremely effective pain control technique, with a surprisingly high number of minor adverse events but safe with regard to serious adverse events. The local research ethics committee approved and registered this study on 16 May 2022 (registration number: S66594).

Improving Quality of Life With Dynamic Compression Bracing in Patients With Pectus Carinatum

BackgroundPatients with pectus carinatum have lower quality of life and self-esteem than their peers. We assessed the impact of dynamic compression system bracing on quality of life in patients with pectus carinatum. MethodsWe conducted a prospective cohort study on patients aged 10–21 years. We assessed quality of life using the Child Health Questionnaire-87, the State-Trait Anxiety Inventory-6, the World Health Organization Quality of Life-BREF, the 36-Item Short Form Survey, and the Single-Step Questionnaire adapted for pectus carinatum. ResultsBetween March 2013 and March 2016, 225 patients treated with dynamic compression system bracing were included. Patients showed improvements across the overall scores of the 36-Item Short Form Survey (Δ7.7 (2.9–12.4)), Single-Step Questionnaire (Δ4.1 (2.0–6.3)) and three out of four World Health Organization Quality of Life-BREF domains (physical health (Δ8.7 (3.7–13.7)), psychological health (Δ11.8 (6.1–17.5)), environment (Δ5.7 (0.2–11.3))). No changes across the Child Health Questionnaire-87 overall score were observed (Δ5.5 (−0.5–11.5)). Most improvement occurred within six to twelve months after treatment initiation, stabilizing thereafter. Anxiety scores on the State-Trait Anxiety Inventory-6 did not improve (Δ0.5 (−0.1–1.2)). Scores on physical complaints, pain, psychological health and self-esteem/self-image improved across all questionnaires. In contrast to the successfully treated group, the unsuccessfully treated group showed no improvement on any of the questionnaires. Most patients (87.2 %) would choose bracing again, 94.9 % of patients were satisfied with the treatment. ConclusionsDynamic compression system bracing improves quality of life, reduces physical complaints and pain and boosts psychological health and self-esteem in patients with pectus carinatum.

Neither Carinatum nor Excavatum: Insights Regarding the Novel Postprematurity Thoracic Dysplasia and Traditional Harrison Grooves

Background/purposeIn recent years, a new chest wall malformation (CWM), the postprematurity thoracic dysplasia (PPTD), was reported. It was characterized as an inward deviation of the anterior tips of the 6th ribs and a personal history of prematurity and respiratory distress. At the moment, PPTD's prevalence and etiology are yet to be defined. Thus, we aimed to explore and identify patients with a compatible morphology within a large cohort of patients with CWM. MethodsRetrospective analysis of electronic health records including medical photographs of 4640 consecutive patients with chest wall malformations at our Pectus Clinic between 2002 and 2023. Patients presenting with bilateral inward curvature of the 6th to 8th ribs were included. Among the variables analyzed, the history of preterm birth and ventilatory support requirement were evaluated. ResultsFrom 2069 patients with non-excavated deformities, 29 (1.4%) patients presented a PPTD morphology. After excluding 12 for incomplete records, we found that 9 patients (52.9%) had a positive history of prematurity (group PT) and 8 (47.1%) didn't (group T). However, both groups showed a high incidence of respiratory comorbidities (PT: 89% vs. T: 76%, p = 0.91). The median age of deformity onset was 12 months (IQR 2; 12) and 24 months (IQR 1; 60) for the PT and T groups, respectively (p = 0.37). Both groups showed a low incidence of family history of CWM (PT: 38% and T: 14%, p = 0.57). ConclusionsIn this retrospective study including 4640 patients with chest wall malformations, the presence of a phenotype described as postprematurity thoracic dysplasia was similarly distributed among patients with or without a history of prematurity. We hypothesize that this condition, different from pectus excavatum and carinatum, might correspond to the Harrison grooves, a deformity whose etiology was profusely discussed in the medical literature from the first half of the 20th century. Type of studyComparative retrospective study. Level of evidenceIII.

Sternal compression before bar placement: Enhancing cosmetic outcomes and facilitating minimally invasive correction of pectus carinatum

Sternal compression before bar placement: Enhancing cosmetic outcomes and facilitating minimally invasive correction of pectus carinatum

Diminished GALNS activity in induced pluripotent stem cells of mucopolysaccharidosis IVA caused by compound p. S162Y and p. C165F mutation.

Mucopolysaccharidosis (MPS) IVA is a lysosomal storage disorder caused by mutations in the gene encoding the galactosamine (N-acetyl)-6-sulfatase (GALNS) enzyme. Children with MPS IVA usually develop pectus carinatum, genu valgum, and multiple skeletal abnormalities. To establish a patient-derived induced pluripotent stem cell (iPSC) disease model to investigate the effects of two GALNS missense mutations. The medical history and clinical manifestations of a patient with MPS IVA were first inspected. The effects of the identified GALNS mutations were predicted through bioinformatic analysis. iPSCs were then generated by using Sendai virus to introduce Yamanaka reprogramming factors to urinary cells isolated from the patient. The pluripotency, karyotypic integrity, genetic mutations, and differentiation ability of the iPSCs were tested. The effects of the GALNS mutations were further experimentally characterized using patient-derived cells. The patient exhibited a typical MPS IVA phenotype. Enzyme replacement therapy could not correct her skeletal abnormalities. GALNS c.485C>A (p.S162Y) and c.494G>T (p.C165F) mutations, inherited from her father and mother respectively, were identified in the patient. These two mutations were predicted to disturb the hydrophobic core of the GALNS catalytic domain. Patient-derived iPSCs were successfully generated, and further characterization indicated that the two missense mutations significantly diminished GALNS activity without affecting its amount at both the RNA and protein levels. We established a novel clinically relevant MPS IVA disease model that will be useful not only for investigating the pathogenic mechanisms of MPS IVA variants but also for drug screening and preclinical evaluation of novel therapies.

The individualized treatment for minimally invasive repair of pectus carinatum in adolescent: a single center’ s retrospective study

BackgroundPectus carinatum (PC) mainly present at the growth spurt time of the early teenage years or the puberty. Poor outer appearance is a major reason for seeking help for surgeons to increase self-confidence and self-esteem. At present, minimally invasive repair (MIR) is one of effective ways to correct the chest wall deformity. Therefore, there is great practical significance to conduct clinical research on MIR about the adolescent PC.MethodsWe applied Abramson procedure in PC group or we applied Abramson procedure and Nuss procedure in PC/PE group. We retrospectively reviewed the results of 41 cases who underwent the surgical correction at our department from January 2020 to April 2023.ResultsAll the procedures were successfully done without severe complications. The median operation Time was 80 min in PC group while was 130 min in PC/PE group. The median LOS were 4 days in PC group while 5 days in PC/PE group. The median compression depth was 32 mm in PC group while 12 mm in PC/PE group. Postoperatively, there are some complications. All Pneumothorax patients being treated conservatively were found in 9 patients in two groups. One patient suffered overcorrection after operation. There were 3 patients suffered steel wires breakage in two groups. One patient reoperation postoperatively for the dislocation of the bar secondary to steel wires breakage.ConclusionsThe Abramson procedure or Abramson procedure and Nuss procedure have good short-term results in repair PC and PC/PE. Select one or two procedures should be done individually based on whether the lower plane over depressed after Abramson procedure.

Clinical characteristics and early results of the modified abramson technique to treat pectus carinatum at Viet Đuc Hospital from 2020-2023

Pectus carinatum is the second most common congenital anterior chest wall deformity. This study describe the clinical, paraclinical characteristics and evaluate the early results of the modified Abramson technique to treat pectus carinatum at Viet Duc Hospital. This is a retrospective descriptive study, in all cases diagnosed with pectus carinatum and underwent surgery according to the modified Abramson technique at Viet Duc Hospital from 1/2020 to 12/2023. In 134 patients, 131 were male, the mean age 13.6 ± 1.98 years. All patients presented with cosmetic complaints, and 85.2% patients have BMI &lt;18.5. 94/134 cases (70.2%) had symmetrical deformities, 37/134 cases (27.6%) had asymmetrical deformities, and 3 cases (2.2%) had mixed pectus deformities. The average Haller index is 2.1 ± 0.3. All patients were operated using a bar, fixed at both ends with steel thread. Operative time was 33.6 ± 7.76 minutes, and hospital stay was 5.3 ± 0.76 days (4–7 days). There was no significant early postoperative complications. Conclusion: clinical examination and computed tomography play an important role in diagnosing pectus carinatum. The modified Abramson technique is a minimally invasive surgical method for pectus carinatum patients with short operative time, hospital stay, and the first step gives good treatment results. TRANSLATE with x English Arabic Hebrew Polish Bulgarian Hindi Portuguese Catalan Hmong Daw Romanian Chinese Simplified Hungarian Russian Chinese Traditional Indonesian Slovak Czech Italian Slovenian Danish Japanese Spanish Dutch Klingon Swedish English Korean Thai Estonian Latvian Turkish Finnish Lithuanian Ukrainian French Malay Urdu German Maltese Vietnamese Greek Norwegian Welsh Haitian Creole Persian TRANSLATE with COPY THE URL BELOW Back EMBED THE SNIPPET BELOW IN YOUR SITE Enable collaborative features and customize widget: Bing Webmaster Portal Back

Chromosomal instability in a patient with ring chromosome 14 syndrome: a case report

BackgroundRing chromosome 14 syndrome is a rare disorder primarily marked by early-onset epilepsy, microcephaly, distinctive craniofacial features, hypotonia, intellectual disability, and delay in both development and language acquisition.Case presentationA 21-year-old woman with a history of epileptic seizures since the age of 1.5 years presented with distinctive craniofacial features, including a prominent and narrow forehead, sparse and short eyebrows, palpebral ptosis, horizontal palpebral fissures, a broad nasal bridge, a prominent nasal tip, a flat philtrum, hypertelorism, midfacial hypoplasia, horizontal labial fissures, a thin upper lip, crowded teeth, an ogival palate, retrognathia, and a wide neck. Additional physical abnormalities included kyphosis, lumbar scoliosis, pectus carinatum, cubitus valgus, thenar and hypothenar hypoplasia, bilateral hallux valgus, shortening of the Achilles tendon on the left foot, and hypoplasia of the labia minora. Chromosomal analysis identified a ring 14 chromosome with breakpoints in p11 and q32.33. An aCGH study revealed a ~ 1.7 Mb deletion on chromosome 14qter, encompassing 23 genes. Genomic instability was evidenced by the presence of micronuclei and aneuploidies involving the ring and other chromosomes.ConclusionThe clinical features of our patient closely resembled those observed in other individuals with ring chromosome 14 syndrome. The most important point was that we were able to verify an instability of the r(14) chromosome, mainly involving anaphasic lags and its exclusion from the nucleus in the form of a micronucleus.

Bi-valve braces for treatment of pectus carinatum in teenagers: impact on patients quality of life

BackgroundPectus carinatum may be a major issue for adolescent patients, as chest-wall malformations can have a negative impact on body image and induce self-esteem disorders. Bracing has become the first line treatment for adolescent patients whose anterior chest wall is still flexible. The main obstacle is the tolerability of the bracing device along with the patient’s compliance to the device. The aim of this study was to examine the quality of life of adolescent patients treated with bracing to correct pectus carinatum in our institution. From November 2021 to July 2023, 28 patients with pectus carinatum were treated with bracing therapy in our chest wall deformities unit. We used a bivalve brace, manufactured with computer-aided design. All patients were asked to answer a multiple-choice questionnaire designed to assess how bracing therapy impacts their daily life, treatment compliance, and overall satisfaction. Patients who wore the brace for at least 3 months, and at least 5 days per week and 10 h per day, are defined as compliant with the bracing protocol.ResultsThe mean age of the patients was 14 ± 1.36 years. Patients wore the brace for an average of 10.1 ± 6.9 months in the compliance group (n = 18), and 2.2 ± 2.5 months in the non-compliance group (n = 10). Our results show that compliant patients are fairly satisfied of the comfort of the brace, with a limited impact of the device on their daily activities and social life. However, non-compliant patients report the following dissatisfactions regarding the brace: higher pain scores, difficulty in breathing, and issues sleeping with the brace. The compliant patients noted a significant improvement in the appearance of their chest, and were satisfied with the appearance of their chest. The non-compliant patients did not notice any changes.ConclusionsCompliant patients treated with a bi-valve brace for pectus carinatum seem to have a good quality of life with a limited impact on their daily life and social activities. The bi-valve brace also seems to be an effective therapy in compliant patients.

Bracing of children with pectus carinatum: A literature review

BACKGROUND: Conservative treatment of children with carinatum is currently an urgent and unresolved problem despite the wide range of techniques available. Conservative treatment stops the progression of deformation and reduces the degree of deformation or eliminates it. Methods of evaluating treatment results include clinical (photo–video assessment, questionnaire survey, and chest measurements) and instrumental diagnostics (radiography, computed tomography [CT], and 3D scanning); however, no method of correction and orthosis that would consider all the tasks of conservative treatment, method of evaluating conservative treatment, and uniform protocol have been established. AIM: This study aimed to analyze the literature containing information on the conservative treatment of patients with pectus carinatum. MATERIALS AND METHODS: The study presents the results of a search in PubMed, Cochrane, and eLibrary using combinations of operators and keywords. A total of 54 foreign and domestic sources were extracted, and the search was not limited retrospectively. RESULTS: Orthosis is effective and positively perceived by doctors and patients. The most modern designs in the treatment of carinatum included dynamic orthoses FMF-DCS and Braceworks (Calgary). Recent studies have reported a strong correlation between 3D scanning and CT to assess treatment outcomes. However, no single option for conservative correction of deformity allowed for achieving all treatment goals. The assessment of the effectiveness of orthosis using photos, videos, and questionnaire, and chest measurement is subjective. CONCLUSIONS: The disadvantages of prosthetics determine the need to develop a clear treatment algorithm, methodology, and objective assessment of treatment outcomes, requiring further research.

Spinal posture, mobility, and position sense in adolescents with chest wall deformities: a comparison of pectus excavatum, pectus carinatum and healthy peers

PurposeThe study aimed to compare spinal posture, mobility, and position sense in adolescents with pectus excavatum (PE), pectus carinatum (PC), and healthy control (HC).Methods22 with PE, 22 with PC, and 21 HC were included in the study. The spinal posture (thoracic kyphosis, lumbar lordosis, pelvic tilt, thoracic, lumbar, pelvic lateral tilt angles) and mobility (thoracic, lumbar, hip/sacral, and overall, in the sagittal and frontal plane) with the spinal mouse, and spinal position sense (repositing errors) with the inclinometer were assessed.ResultsThe thoracic kyphosis angle of PE and PC was higher than in HC (p < 0.001; p = 0.001). Hip/sacral mobility in the sagittal plane was lower in the PE and PC than control, respectively (p < 0.001; p < 0.001). Overall sagittal spinal mobility (p:0.007) and hip/sacral mobility in the frontal plane (p:0.002) were lower in the PC than in HC. Overall frontal spinal mobility was lower in the PE and PC than in HC (p:0.002; p:0.014). The PE and PC repositing errors were higher (p < 0.001; p:0.014).ConclusionThe study found that adolescents with PE and PC had decreased spinal mobility, spinal alignment disorders, and a decline in spinal position sense. It is important not to overlook the spine during physical examinations of adolescents with chest wall deformities. In clinical practice, we suggest that adolescents with chest deformities should undergo a spine evaluation and be referred for physical therapy to manage spinal disorders.

The sandwich technique used for correction of pectus carinatum combined with Harrison sulcus

We aimed to investigate the feasibility of the sandwich technique to treat pectus carinatum combined with Harrison sulcus. We retrospectively analysed the clinical data of 38 paediatric patients with pectus carinatum combined with Harrison sulcus treated from June 2015 to October 2022. All the patients underwent surgery using the sandwich technique. The surgical conditions and postoperative outcomes of the patients and the satisfaction score of family members were analysed. Overall, the patients had a mean duration of surgery of 179.05 ± 36.01 min, intraoperative blood loss of 10.03 ± 2.77 mL, postoperative hospital stay of 6.89 ± 0.73 days, and postoperative satisfaction score of 89.4 ± 4.6. The incidence of surgical complications was 7.89%. The internal fixation stents were removed in 22 patients, and there was no recurrence during a follow-up 371.4 ± 6.3 days post-stent removal. These results were satisfactory. The use of the sandwich technique to treat this condition does not reduce the volume of the thorax after the procedure and results in an aesthetically pleasing incision, less complications, and fast postoperative recovery. Thus, it is a safe and effective method that is worthy of being promoted for clinical application.

Spinal deformities and other orthopedic disorders in children with pectus carinatum

Spinal deformities and other orthopedic disorders in children with pectus carinatum

Assessment of Cardiac Anomalies in Pediatric Patients with Pectus Carinatum

Background: Pectus carinatum (PC) is an anterior thoracic visible deformity manifested by protrusion of the sternum. It can be an isolated chest deformity or it can be seen together with genetic pathologies. Methods: We analyzed the cardiac findings of 40 children who were diagnosed with pectus carinatum at Kahramanmaras Sutcu Imam University’s Thoracic Surgery Outpatient Clinic between 1 February 2021 and 1 February 2022 and 97 healthy controls in similar age groups. Results: The Pectus Carinatum patients had higher rates of cardiac malposition, MVP (Mitral valve prolapse), MI (mitral insufficiency), TVP (tricuspid valve prolapse), CHD (congenital heart disease), and aortic valve pathology. Conclusıons: Our study showed that the prevalence of cardiac pathologies in the pediatric PC patients was higher than that in the control group consisting of healthy children. Thus, it may be recommend the referral of pediatric PC patients to cardiology outpatient clinics for the early diagnosis of potential cardiac pathologies.

Respiratory muscular strength in children with mucopolysacaridosis: comparison with predictive equations.

Mucopolysaccharidoses (MPS) are rare metabolic diseases that impair respiratory function leading to respiratory failure. This study aimed to compare maximal inspiratory and expiratory pressures (MIP and MEP) obtained in children with MPS and compare with predicted values from previous studies involving healthy children. This is a cross-sectional study, in which the chest deformity was evaluated; MIP, MEP through digital manometer, and lung function through spirometry. MIP and MEP were compared with five different predict equations and with a control group of healthy children. Agreement between respiratory muscle weakness regarding absolute values of MIP and MEP in relation to predictive values by the equations included in the study were assessed by Kappa coefficient. MPS group was composed of 22 subjects. 45.5% had pectus carinatum, 36.4% pectus excavatum, and presented lower MIP (37.14±36.23 cmH<inf>2</inf>O) and MEP (60.09±22.3 cmH<inf>2</inf>O) compared with control group (22 healthy subjects) (MIP: 91.45±35.60; MEP: 95.73±22.38). Only the MEP equations proposed by Tomalak et al. were close to those found in our MPS children (P=0.09). In the MPS group it was observed a weak agreement between inspiratory weakness through absolute and predicted values in only two equations: Tomalak et al. and Domenèch-Clar et al. (for both: k=0.35, P value =0.03); and for MEP a moderate agreement was found using all predictive equations. In MPS children MRP data should not be normalized using the reference equations for healthy ones, is more coherent to longitudinally follow absolute pressures and lung volumes in this group.

The role of 3D printing in chest wall reconstruction.

Technology is advancing fast, and chest wall surgery finds particular benefit in the broader availability of three-dimensional (3D) reconstruction and printing. An increasing number of reports are being published on the use of these resources in virtual 3D reconstructions of chest walls in computed tomography (CT) scans, virtual surgeries, 3D printing of real-size models for surgical planning, practice, and education, and of note, the manufacture of customized 3D printed implants, changing the fundamental conception from a surgery that fits all, to a surgery for each patient. In this review, we explore the evidence published on simple chest wall reconstruction, including the use of 3D technology to assist in the improvement of the repair of the most frequent chest wall deformities: pectus excavatum and carinatum. Current studies are oriented to the automatization and customization of transthoracic implants, as well as education on real-size models. Next, we investigate the implementation of 3D printing in the repair of complex chest wall reconstruction, comprised of infrequent chest wall deformities such as pectus arcuatum and Poland syndrome. These malformations are very heterogeneous resulting in a high degree of improvisation during the surgical repair. In this setting, 3D technology plays a role in the standardization of a process that contemplates customization, concepts that may seem contradictory. Finally, 3D printing with biocompatible materials is rapidly becoming the first choice for the reconstruction of wide chest wall oncological resections. In this work, we review the first and most important current publications on the subject.

Вторинні порушення форми грудної клітки після корекції лійкоподібної деформації за методом Nuss

Aim - studying the influence of the elasticity of the chest on the formation of secondary pectus carinatum and secondary protrusion of costal arches after Nuss procedure in order to determine the risk groups for the occurrence of data of secondary postoperative deformations in order to optimize a surgical intervention in these groups . Materials and methods. We analysed the data of 6 patients with pectus carinatum and 5 children with rib arches protrusion, which were formed as a result of Nuss funnel chest correction. The elasticity of the chest was studied by determining the ratio of its circumference during maximum inhalation and exhalation (mobility index - MI) as well as the ratio of the distance between the support plane in the supine position and the front surface of the chest in a neutral position and at the moment of compression (compliance index - CI). To calibrate the indicators, the data were calculated in a group of 30 healthy children. Depending on the obtained values, 3 degrees of chest elasticity are distinguished: high, medium and rigid chest. Results. 3 degrees of chest elasticity were determined in children without chest deformity: high (MI - 9.1±0.9%; CI - 28.1±1.8%), medium (MI - 6.8±0.2%; CI - 22.7±2.1%) and a low or rigid chest (MI - 4.9±0.2%; CI - 15.6±1.8%). The following data were recorded in patients with secondary deformities: 80% of patients with secondary pectus carinatum had a high degree of elasticity of the chest, 20% - average degree. In 83.33% of cases, patients with secondary rib arches protrusion had a high degree of elasticity of the chest, and 16.67% had an average degree. Secondary rib arches protrusion in 80% of cases is combined with the use of one horizontal bar. All cases of occurrence of secondary deformations are associated with a symmetric local type of funnel chest with or without deformations of costal arches or with an asymmetric local type. Conclusions. Using the Nuss operation technique with short plates in patients in the period of rapid growth with a high degree of elasticity of the chest with type IA and IIA1 of funnel chest can reduce the risk of secondary pectus carinatum; and in cases of high level risk of secondary protrusion of the rib arches the "flare buster" technique can help avoid this complication. The vast majority of secondary deformations are prone to spontaneous regression. The research was adhered to the principles of the Declaration of Helsinki. The research protocol was agreed by the Local Ethics Committee of the mentioned institution. The patients' informed consent was obtained for the study. No conflict of interests was declared by the authors.

Poland syndrome: a case report

Poland syndrome is characterized by the absence or hypoplasia of other chest muscles: small pectoral muscle, anterior tightened muscle, grandorsal muscle, and deltoid muscle. Anomalies of the thoracic cage: agenesis or hypoplasia of one or more costal segments, pectus carinatum, excavatum, clavicular hypoplasia, pulmonary herniation. Abnormalities of the mammary region: agenesis or hypoplasia of the breast, areola and nipple. The reported incidence of Poland’s syndrome ranges from 1 in 7,000 to 1 in 100,000. Males are affected more frequently by a 2:1 to 3:1 ratio Poland’s syndrome has also been diagnosed in 1 of 19,000 mammograms. The right side of the body was found to be involved in 60% to 75% of patients - associated malformations: genitourinary malformations, cardiac malformation, hepatic and biliary tract malformations. Case report Our case involves a 7-month-old boy with no medical history of cardiac or respiratory complaints. This is a male child with around 65cm height, and 7kg weight. There is no family history of consanguineous marriage and no family history of congenital anomalies. All siblings are alive and healthy. The child’s milestones are within normal limits. There is aplasia of the right sternocostal head and clavicular heads and abdominal head of the pectoralis major muscle, there is a hypoplasia of the right nipple and breast and absence of subcutaneous fat on the same side. Ribs are visible on the right chest wall. Physical examination shows no history of hand and digit anomaly and no rib anomalies. Chest wall asymmetry with right chest wall showing depression on axillary floor. The anterior axillary fold is absent. On attempting abduction of the shoulder, it shows the absence of the sternocostal head of the pectoralis major.