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Pre-operative three-dimensional surface reconstructions of the same 24-year-old patient showing sternal depression and tilting.
Source publication
Congenital chest wall deformities are caused by anomalies of chest wall growth, leading to sternal depression or protrusion, or are related to failure of normal spine or rib development. Cross-sectional imaging allows appreciation not only of the involved structures, but also assessment of the degree of displacement or deformity of adjacent but oth...
Citations
... Due to the rarity of the disease and the variation in the degree of the defect, the management protocols are confined only to some case reports. Few centers have described the use of repair of the sternal defect using synthetic mesh, porcine acellular dermal matrix, etc., but there remains a high risk of sternal infection and mediastinitis [4]. Early intervention is also preferred to support respiratory function and it is of utmost importance to maintain adequate preload, right heart function, ventilation and oxygenation to account for any alteration in lung compliance [5]. ...
Congenital absence of sternum is a rare malformation of the anterior chest wall that needs surgical correction to avoid life-threatening complications as a consequence of such defect. It results from either partial or complete failure of fusion of mesenchymal strip during in utero organogenesis. The absence of sternum entails the risk of trauma to the mediastinal structures and other life-threatening complications. This defect is evaluated by a thorough clinical examination and computed tomography imaging of the thoracic cage to plan the surgical procedure. Early repair of the defect when the thoracic cage is still compliant yields the best result.
... The crucial part of the program is during the first year of treatment, whereby patients need to be followed closely at consultation. To date, the best criterion to describe pectus severity is the Haller index (HI) [12,13]. Its calculation requires cross-sectional images, which are not feasible at each consultation. ...
... The HI has been favoured as the main criterion, even for early evaluation [16]. The HI and EHI are commonly described as criteria for PE and rarely for PC [12]. The correlation with patient satisfaction such as reduction of anxiety and improved body satisfaction is expected [11], even though the correlation with morphologic indexes was not performed. ...
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). The main objectives were to assess the effectiveness of the treatment during the first year and to compare the HI determined by MRI to the EHI evaluated with 3D scanning and external measurements. The HI determined by MRI was compared to the EHI evaluated with 3D scanning and external measurements at M0 and M12.
Results:
118 patients (80 PE and 38 PC) had been referred for pectus deformity. Of these, 79 met the inclusion criteria (median age 13.7 years, 8.6-17.8). There was a statistically significant difference in the external measurements of the depth for PE between M0 and M12: 23.0 mm ± 7.2 vs 13.8 mm ± 6.1, respectively; p < 0.05 and for PC 31.1 mm ± 10.6 vs 16.7 mm ± 8.9, respectively; p < 0.01. During this first year of treatment, the reduction in the external measurement increased more rapidly for PE compared with PC. We found a strong correlation between the HI by MRI and the EHI by 3D scanning for PE (Pearson coefficient = 0.910, p < 0.001) and for PC (Pearson coefficient = 0.934, p < 0.001). A correlation between the EHI by 3D scanning and the external measurements by profile gauge was found for PE (Pearson coefficient = 0.663, p < 0.001) but not for PC.
Conclusions:
Excellent results were observed as soon as the sixth month for both PE and PC. Measurement of protrusion is a reliable monitoring tool at clinical consultation but caution is required for PC as it does not appear to be correlated to the HI by MRI.
... Lactante masculino con deformación torácica importante, dada por la presencia de pectum excavatum, que suele presentarse en el tercio inferior del esternón y con los cartílagos centrales hundidos, que en muchas ocasiones pueden estar presentes desde el nacimiento, el 21% presentan escoliosis y el 11% tienen historia familiar. 1 La presencia de pectum excavatum favorece la presencia de patrones respiratorios de tipo restrictivo, lo que ocasiona, junto con el resto de los síntomas respiratorios, manifestaciones de mayor compromiso ventilatorio. Esta deformación torácica afecta, a su vez, a los músculos respiratorios, impidiendo que alcancen su máxima contractilidad, reduciendo de manera significativa (hasta menos del 50% del valor esperado) de las presiones máximas inspiratorias y espiratorias, además de que no se presenta la movilidad torácica normal durante los movimientos respiratorios, mostrando mayor respiración de tipo abdominal. ...
Niño de un año de edad al fallecimiento con daño neurológico por asfixia perinatal y malformaciones esqueléticas (pecho excavado y pie varo equino), que cursó con numerosos cuadros de infección respiratoria y broncoaspiración, lo que requirió atención hospitalaria en múltiples ocasiones. Fue visto por múltiples especialistas logrando solamente una muy leve recuperación en tratamiento en rehabilitación y falleció de forma inesperada en su domicilio.
... This anomaly is the most common second deformity of the chest wall. This anomaly, seen at a rate of 0.1%, is more common in males [7,17,23]. In our study, pectus carinatum prevalence was 0.45%. ...
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. Descriptive statistics were performed with the distribution of anomalies. Comparisons were made between the genders and the directions.
Results: A prevalence of 18.57% rib variation was observed. Women had 1.3 times more
variation than men. Although there was a significant difference in the distribution of anomalies by gender (p=0.000), there was no difference in terms of anomaly direction (p>0.05). The most common anomaly was the hypoplastic rib, followed by the absence rib. While the incidence of the hypoplastic rib was close in women and men, 79.07% of the absence rib was seen in women (p<0.05). The study also includes a rare case of bilateral first rib foramen. At the same time, this study includes a rare case of rib spurs extending from the left 11th rib to the 11th intercostal space.
Conclusions: This study demonstrates detailed information about congenital rib anomalies in the Turkish population, which may vary between people. Knowing these anomalies is essential for anatomy, radiology, anthropology, and forensic sciences.
... Therefore, internal imaging examinations are indispensable in the diagnosis and follow-up of PE. Routine examination of patients with PE by computed tomography (CT) can completely present the chest wall structure and accurately assess the severity of the condition, guiding treatments such as functional exercise, vacuum bells, and surgical intervention (4). However, when assessing the severity of PE, the HI and CI indices are only measured at the cross-section of the deepest sternal depression observed by multiple CT scan slices. ...
Introduction
Objective assessment of the severity of pectus excavatum (PE) mainly depends on internal imaging examination, which poses radiation exposure risks and high financial costs. Our study explores the feasibility of caliper-based external measurements of the body surface to assess PE severity.
Materials and methods
Patients with PE aged 4–18 years who underwent both internal imaging examinations and external measurements were chosen for the study. Overall, 176 patients underwent surgery and 21 underwent regular observation. The Haller index (HI) and correction index (CI) were used to derive the external measurement indices, HI-caliper and CI-caliper. Receiver-operator characteristic analysis provided the optimal cut-off values and compared the diagnostic values of HI-caliper and CI-caliper. Spearman's correlation coefficient and Cohen's kappa coefficient were used to analyze the correlation and consistency between HI-caliper or CI-caliper and HI-CT or CI-CT, respectively. Also, a paired samples t -test was used to compare the differences of HI-caliper or CI-caliper before and after surgery.
Results
HI-caliper and CI-caliper measurements had strong correlations with HI-CT and CI-CT results (rs = 0.70, p < 0.001; rs = 0.69, p < 0.001), respectively. The optimal cut-off values of HI-caliper and CI-caliper were 1.83 (sensitivity = 0.841, specificity = 0.905) and 12% (sensitivity = 0.881, specificity = 0.857), exhibiting comparable diagnostic values with HI-CT and CI-CT. HI-caliper > 1.83 or CI-caliper > 12% had medium intensity consistency with HI-CT ≥ 3.25 or CI-CT ≥ 28% ( k = 0.545, 95% confidence interval: 0.374–0.716, p < 0.001). The HI-caliper and CI-caliper values were significantly different before and after surgery.
Conclusion
Caliper-based external measurement is a feasible method to screen patients who require surgical intervention and for monitoring the progression of PE severity.
... In contrast to pectus excavatum, carinatum will demonstrate increased AP diameter of the chest. Using the ratio of transverse and AP diameter of the chest (Fig. 3), the severity can be calculated, an abnormal index ranges from 1.4 to 1.96, a value less than 1.8 usually quoted as requiring corrective surgery (Fig. 3) [17]. ...
Abnormalities of the sternum and adjacent structures are an uncommon presentation in the paediatric population and can have a variety of benign and malignant causes, including normal and developmental variants of the chest wall. Although there is overlap with adults, many sternal abnormalities are unique to the paediatric population. Following clinical examination, radiography is usually the first type of imaging used; however, it is limited and often ultrasound and cross-sectional imaging are needed for further assessment. An understanding of the normal anatomy is important; however, this can be challenging due to the varied appearances of age-related changes of the sternum. The purpose of this article is to familiarize the general paediatric radiologist with the expected anatomy and imaging findings of the developing sternum, anatomical variants and pathology of the sternum and adjacent structures encountered in this group of patients.
... Similarly to PC treatment, PC diagnostics has undergone substantial changes. Until recently, CT examination of the chest was considered as a standard for morphological assessment of deformity and the Haller Index was subsequently calculated (a ratio of the maximum internal transverse diameter of the chest divided by the minimum anteroposterior diameter) [26]. This procedure (connected with X-ray use) can be replaced by anthropometric examination, as demonstrated by Štefánková et al. in pectus excavatum patients, while the correlation analysis between the TI and the Haller Index shows a strong inverse dependence [17]. ...
Objectives: Brace treatment in children with pectus carinatum has become the method of choice during the last decade. The authors evaluate the role of anthropometric measurements in diagnostic and treatment processes.
Methods: A prospective study, analysing a compressive brace treatment for pectus carinatum, performed between January 2018 and September 2020. Demographic data, anthropometric dimensions and indexes of the chest, data connected to an orthosis usage, as well as ongoing treatment outcomes were analysed.
Results: Forty-seven consecutive patients aged between 10 to 18 years with pectus carinatum were prescribed a compressive brace. Thirtynine of them (83 %) reached clinically positive results while wearing the orthosis for 6 ± 3 months. An improvement in the sagittal chest diameter was 0.5 cm – 2.8 cm (mean 1.0 cm ± 0.5 cm) and an improvement of the Thoracic Index was 0.8 % – 25.1 % (6.4 % ± 4.5 %) by using the brace on average for (6 ± 2) hours a day.
Conclusion: Clinical anthropometric measurements can evaluate the dimensions of chest wall and treatment progress in patients with pectus carinatum precisely and thus replace the need for more complex examinations requiring X-rays.
... While typically cosmetic, severe deformities have been associated with pain and restrictive lung disease [22]. Pectus excavatum is more common and characteristically manifests on frontal chest radiographs as an obscured right heart border with leftward cardiac rotation and displacement by the posteriorly angulated sternum [61]. In cases of severe thoracic deformity or decreased cardiac or respiratory reserve, surgical reconstruction may be warranted, and a CT thorax may be useful for surgical planning. ...
... The clavicles are also broad and short in morphology, and the glenoid fossae are dysplastic Pediatr Radiol osteocartilaginous resection and manipulation through the Ravitch procedure. In these cases, postoperative imaging is valuable to assess the location of the bar and to detect postoperative complications such as pneumothorax [61]. ...
Pathologies involving the ribs are diverse in nature, including entities specific to the pediatric population as well as shared pathologies with adults. These can be either localized within or adjacent to the rib, but may also cause rib alteration as a component of a systemic process. To better understand these disorders, we discuss several common rib pathologies in the context of their clinical presentation and pertinent imaging findings. In addition, we review the imaging modalities that may be used to evaluate the ribs. Encompassing both the clinical and imaging aspects of pediatric rib pathologies, this review aims to increase pediatric and musculoskeletal radiologists’ awareness of the spectrum of disease and how to leverage a pattern-based approach.
... Yaklaşık 400 doğumda bir görülür ve erkeklerde daha sıktır [1][2][3][4]. Bu deformite, çoğunlukla konjenital olarak görülürken vakaların %15 kadarında ergenlik süresince gelişimsel olarak sonradan da ortaya çıkabilir [5]. Etyolojisi tam olarak bilinmese de alt kostal kıkırdakların aşırı büyümesi en makul teorilerden biridir [1,5]. ...
... Bu deformite, çoğunlukla konjenital olarak görülürken vakaların %15 kadarında ergenlik süresince gelişimsel olarak sonradan da ortaya çıkabilir [5]. Etyolojisi tam olarak bilinmese de alt kostal kıkırdakların aşırı büyümesi en makul teorilerden biridir [1,5]. Ayrıca Marfan, Ehlers-Danlos ve Noonan sendromu gibi bazı bağ dokusu hastalıkları ile birlikteliğinin sıklığı muhtemel bir genetik geçiş varlığını da desteklemektedir [3,5]. ...
... Etyolojisi tam olarak bilinmese de alt kostal kıkırdakların aşırı büyümesi en makul teorilerden biridir [1,5]. Ayrıca Marfan, Ehlers-Danlos ve Noonan sendromu gibi bazı bağ dokusu hastalıkları ile birlikteliğinin sıklığı muhtemel bir genetik geçiş varlığını da desteklemektedir [3,5]. ...
... Neither a computed tomography (CT) scan nor echocardiography was performed, but preoperative ECG and chest X-ray had shown findings that corresponded to pectus excavatum, which generally include negative P and T waves in V1 on ECG, suggesting aberrant placement of lead V1 due to the dish-like precordia, and a compressed heart (7). Some authors have reported an obscure right heart border and a right-axis shift on chest radiography that are characteristic of severe deformity (8). Pectus excavatum is a congenital anomaly of the chest wall, which occurs approximately in 1 in 400 births. ...
Objective:
Acute pulmonary edema associated with ritodrine hydrochloride is a rare, life-threatening complication, and dose and duration of ritodrine use are closely associated with this pathology. We report a case of acute pulmonary edema associated with short-duration infusion of ritodrine hydrochloride in a patient with pectus excavatum as an underlying factor.
Case report:
A 30-year-old healthy pregnant woman was treated with oral ritodrine for tocolysis between 31 and 35 weeks of pregnancy. At 36 weeks of gestation, she went into preterm labor, with premature rupture of the membrane and breech presentation, and received an infusion of ritodrine hydrochloride for a few hours. Although she was normotensive until labor onset, mild hypertension and proteinuria were recognized. Intraoperatively, a funnel-chest deformity was observed, and she developed postoperative pulmonary edema associated with dyspnea and wet cough and confirmed on chest radiography and arterial gas analysis, and recovered with supportive care.
Conclusion:
Small-dose infusion of ritodrine hydrochloride might cause pulmonary edema in patients with underlying medical problems, including pectus excavatum.
