Book

Chest Wall Deformities and Corrective Procedures

Authors:

Abstract

This book discusses predominantly pectus excavatum and pectus carinatum, its variation and the treatment modalities available, including minimally invasive surgery. This will help to increase awareness of the available treatment options to doctors and healthcare workers but will also help trainee cardiac and thoracic surgeons. It is also an essential reference book for those already practicing. ?Chest wall deformities are a common occurrence, the commonest being pectus excavatum and pectus carinatum. The treatment for these is provided by very few centers and there is a decline in expertise especially in the UK due to rationing. When patients approach their GP they are mostly turned away and asked to live with it due to a lack of awareness and treatment modalities. The Editors have extensive experience in this field and will invite authors from other specialties, i.e. chest physicians, cardiologist, pediatric surgeons, anesthesiologists, pain management team, plastic surgeons and prosthetic departments, to contribute their views and solutions to the book.

Chapters (17)

In Pectus Excavatum, the sternum is cast-down and depressed into a convex shape. The sternal malformation is caused by the extensive growth of the costal cartilages, inserting into the sternal body. The growth allows the cartilages to clump together and further push the sternum inwards. Having an appendicular derivative, the sternum develops from two sternal bar components. The following chapter aims to evaluate the embryological contributing factors to the development of Pectus Excavatum.
Bauhinus produced the cardinal definition of a funnel-form chest in the sixteenth century. He assessed the clinical features of pectus excavatum in a patient suffering with pulmonary compression, dyspnea (shortness of breath) and paroxysmal cough; the symptoms appraised by Bauhinus aided the embellishment of his definition of the deformation. The Nuss procedure to correct the deformity relies on two interventions: the first, to insert the steel bar through bilateral anterior-axillary thoracic incisions and the second, to remove the bar after 3 years.
Among all chest wall deformities Pectus Excavatum (PE) or funnel chest represents the most common congenital chest wall deformity accounting for 90 % of all deformities. The main characteristic is the depression of sternum and lower cartilages (Langer, Herrn JW Wiener med Zeit 49:515, 1880) with an incidence between 1 and 8 per 1000 children.
Pectus Carinatum (PC) or protrusion deformity of the chest wall accounts for 5 % of all chest wall deformities affecting 1 in 2500 live births (Ravitch, Congenital deformities of the chest wall and their operative correction. WB Saunders, Philadelphia, 1977). It is also know as pigeon chest. It can be unilateral, bilateral or mixed and there is predominance in males (Robicsek, Chest Surg Clin N Am 10(2):357–76, 2000).
Congenital chest wall deformities encompass a wide spectrum of conditions and present the patient with varying severities of cardiorespiratory and psychological dysfunction. Surgical intervention has been shown to alleviate symptoms and improve overall psychological wellbeing. Throughout history many different surgical correction techniques have been described for the treatment of congenital chest deformities. One factor that is common to all these surgical techniques is the necessity for detailed pre-operative workup. This chapter aims to explore the imaging modalities that can be used in the pre-operative evaluation of patients with a spectrum of congenital chest wall deformities.
A thoracic index is a formula used to qualify or quantify a thoracic deformity and in some cases, to define strategies within treatment. It is also a diagnostic tool used historically to assess the severity of the defect. There is no definition, classification, or consensus in the literature on which thoracic index is the gold standard. There are also no guidelines regarding cut-off values. This chapter is an effort to put all this together, starting by defining thoracic indexes, proposing a classification and describing them in detail for the first time. The main objective is to find out which are the most commonly indexes used by chest wall malformation experts worldwide, and why. For this reason the present chapter includes a web-based survey made to the aforementioned experts in order to review this issue in detail. Since there is currently no thoracic index without limitations, perhaps the perfect index is a mathematical combination of several different indexes. Perhaps it is one single index still to be discovered. This is the first step to search for a universal thoracic index for surgical – decision making.
Traditional treatment for chest-wall deformities relies upon surgical interventions that aim to increase thoracic function and restore kinetic and structural integrity to halt future chest-wall deformation prevent future deterioration of the chest wall. The Ravitch procedure is the most common intervention and involves subperichondrial resection of the deformed costal cartilages and sternal osteotomy for fixation of the sternum anteriorly. Novel minimally invasive techniques are gaining popularity amongst centers specialising in chest wall reconstruction, such as the Nuss procedure. At our centre we are researching the benefit of patient centered anesthesia on pain management post- Nuss procedure. We are also investigating various different techniques for bar removal and insertion using wire-assisted techniques. All of our research aims to increase the efficacy of minimally invasive corrective procedures.
The modern era of correction of pectus excavatum (PE) started in 1949 by Ravitch. Since several modifications to the technique were published, but it was the standard way to correct PE for long time. Prof Nuss’s minimally technique changed the strategy for correction and seems now to be the standard technique for surgeons who correct PE. The optimal age for surgery is discussed. Most surgeons prefer that the patient is in the beginning of the puberty so the bar system is in situ through the growth spurt. It looks like that this decreases the recurrence rate. At this age the patients are also aware of the restrictions which are in the beginning of the treatment. But recently it has been offered to patient up to 40 years of age. Bar removal is done 3 years after correction and is a day surgery project. In most cases it is only necessary to open the incision where you have the stabilizer if you use the short bar technique. The complication are few in experienced hands. Most of the patients get a very beautiful result and are very satisfied with the operation.
Pectus Carinatum is the second mostly encountered congenital chest wall deformity following Pectus Excavatum. Deformity becomes apparent during puberty, due to active growth; which leads to cosmetic and psychosocial problems. “Minimally Invasive Repair of Pectus Carinatum” gained popularity among surgeons during last decade. In this chapter, we try to explain surgical details, preoperative and postoperative workup period of the deformity. We also present our whole experience about correction of pectus carinatum.
The thoracoscopic placement of Nuss pectus bars for the correction of pectus excavatum is a painful procedure., which poses a challenge for the thoracic anaesthetist. Adequate pain management can expedite post-operative recovery and reduce complications. It may also prevent the development of chronic post-operative pain. Previously thoracic epidural analgesia has been favoured by centres in North America and Europe, but there is tendency to move away from this in favour of a multimodal approach to analagesia, including regional blockade, opiate infusions and patient-controlled analagesia, with non-steroidal anti-inflammatory drugs, paracetamol and other novel analgesics given in addition for their synergistic and opiate sparing effects
Pectus excavatum (PE) is the most common, congenital deformity of the anterior chest wall and represents around 90 % of all anomalies of the anterior chest wall. PE has been very well investigated over the years with a vast amount of studies being produced, still, no consensus has yet been reached on the direct impact of PE on cardiopulmonary function.
There are various other chest wall deformities that are worth discussing. These will be outlined in the following chapter. Jeune Syndrome, also known as Asphyxiating Thoracic Dystrophy (ATD) is a rare autosomal recessive skeletal dysplasia with multiorgan involvement. It was first described by Jeune in 1954 and it affects 1 per 10,0000–13,0000 live births. There are two subtypes of the syndrome with severe subtype being incompatible with life. Poland syndrome (PS) is classified as a chondrocostal chest wall deformity with main clinical manifestation the underdevelopment or absence of the major pectorals muscle. It is a congenital unilateral chest wall deformity that affects both males and females in a ratio of 3:1 and with an incident variation from 1–7,0000 to 1–10,0000 live births. A rarer category of chest wall deformation is pectus arcuatum represents a rare category of chest wall deformities in the family of pectus anomalies and It includes mixed excavatum and carinatum features along a longitudinal or transversal axis resulting in a multiplanar curvature of the sternum and adjacent ribs. Sternal cleft represents a rare idiopathic chest wall deformity caused by a defect in the sternum’s fusion process. It accounts for 0.15 % of all chest wall deformities and there is an association with the Hexb gene. There are four types of sternal clefts according to the classification proposed by Schamberger and Welch in 1990.
Acquired deformities of the chest wall are malformations, which develop due to non-congenital causative factors. Based on etiology, three major categories of acquired chest wall malformations can be distinguished. (1) Primary disease of the chest wall itself can cause deformation of the chest wall. This includes tumors and infections affecting the chest wall with subsequent development of chest wall deformation. (2) The largest group of acquired chest wall deformities are iatrogenic in nature and occur as a result of previous surgical intervention to the chest wall, seen as acquired restrictive thoracic dystrophy or acquired Jeune’s syndrome in young patients following open correction of pectus excavatum deformity. Iatrogenic chest wall deformities may also develop following rip graft harvesting or failed closure of thoracotomies. (3) Post-traumatic deformities are a result of direct or indirect trauma to the torso. This chapter is aimed to provide a comprehensive overview of the spectrum of acquired chest wall deformities and to discuss their pathophysiology, diagnosis and treatment.
Recurrence of pectus excavatum deformities occurs after both open and MIRPE. Recurrence risks are also based on multiple factors and differ based on the initial repair procedure. Identifying the contributing factors to a previous procedure’s failure is critical to proper repair and prevention of another recurrence. Each case must be taken on an individual basis and is contingent on the patient’s anatomy and previous repair technique. A combination of surgical techniques may be necessary in to successful repair some patients.
Adjustment and augmentation of the soft tissues plays an important role in the management of patients with chest wall deformities. Both patients with severe abnormalities that have undergone reconstructive surgery to re-shape the chest wall and those with milder deformities can benefit from such soft tissue augmentation. This chapter discusses a range of autologous and/or implant based techniques that can be useful in these patients to provide an optimum result. The challenges of managing breast asymmetry or hypoplasia in the female patient with a chest wall deformity is also discussed.
Pectus excavatum (PE) and carinatum (PC) are characterized by an abnormal overgrowth of sternal and costal cartilages, which result in a depression or protrusion of the sternum and costal cartilages, respectively. Both chest wall malformations are cosmetic and functional pathologies. Whereas PE is commonly associated to cardiopulmonary dysfunction, PC causes deformation of the entire thoracic cage. PE is generally corrected operatively. In contrast, due to inherent risks of a major surgery, only severe cases of PC are operated. One of the authors (FMH) will describe his 12 years experience with vacuum bells to treat PE patients conservatively. The use of vacuum bells allow significant lift of the ribs and sternum, until definitive correction of cartilage growth takes place. When employed during minimally invasive repair of PE (MIRPE), vacuum bells can also be used as a tool to enhance retrosternal dissection, advancement of the pectus introducer and insertion and flipping of the pectus bar/s. The other author (MMF) will describe his 13 years experience with the FMF® Dynamic Compressor System to treat patients with PC conservatively. When considering results, there should be little doubt that no patient would be selected as a candidate for surgery before trying a non-operative approach. Further evaluation and follow-up studies are still necessary for both conservative approaches, though.
Pectus excavatum is the most frequent congenital anterior chest wall and sternal deformity. The NUSS procedure is a minimally-invasive surgical intervention carried out on patients with the anomaly. The procedure has an extremely high success rate and is proven to benefit the patient’s respiratory and cardiac function. Pectus excavatum patients suffer frequent embarrassment over physical appearance and teasing- 22.8 % patients reported such teasing, with an expected 97.4 % majority of teasing coming from peers. Two patients were chosen, at either end of the age spectrum, and they shared an account of their own experiences.
... A more recent index, the correction index (CI), often better reflects the degree of a funnel chest [7] as it is independent of the width of the thorax. Different metrics exist to quantify thoracic asymmetry, with the asymmetry index (AI) [8] and the eccentricity index (EI) [9] being among the more commonly used. ...
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Objectives: The breathing phase for the determination of thoracic indices in patients with pectus excavatum is not standardized. The aim of this study was to identify the best period for reliable assessments of morphologic indices by dynamic observations of the chest wall using real-time MRI. Methods: In this prospective study, patients with pectus excavatum underwent morphologic evaluation by real-time MRI at 3 T between January 2020 and June 2021. The Haller index (HI), correction index (CI), modified asymmetry index (AI), and modified eccentricity index (EI) were determined during free, quiet, and forced breathing respectively. Breathing-related differences in the thoracic indices were analyzed with the Wilcoxon signed-rank test. Motion of the anterior chest wall was analyzed as well. Results: A total of 56 patients (11 females and 45 males, median age 15.4 years, interquartile range 14.3-16.9) were included. In quiet expiration, the median HI in the cohort equaled 5.7 (4.5-7.2). The median absolute differences (Δ) in the thoracic indices between peak inspiration and peak expiration were ΔHI = 1.1 (0.7-1.6, p < .001), ΔCI = 4.8% (1.3-7.5%, p < .001), ΔAI = 3.0% (1.0-5.0%, p < .001), and ΔEI = 8.0% (3.0-14.0%, p < .05). The indices varied significantly during different inspiratory phases, but not during expiration (p > .05 each). Furthermore, the dynamic evaluation revealed three distinctive movement patterns of the funnel chest. Conclusions: Real-time MRI reveals patterns of chest wall motion and indicate that thoracic indices of pectus excavatum should be assessed in the end-expiratory phase of quiet expiration. Key points: • The thoracic indices in patients with pectus excavatum depend on the breathing phase. • Quiet expiration represents the best breathing phase for determining thoracic indices. • Real-time MRI can identify different chest wall motion patterns in pectus excavatum.
... The sternal depression may restrict thoracic volume and therefore vital capacity; it can have a negative effect in tolerance to physical exercise. PE may also cause cardiac compression reducing cardiac output and further contributing to exercise intolerance [3]. However, these symptoms are rarely disabling and cosmetic features are the most frequent concerns of children with PE [4,5]. ...
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Background: Pectus excavatum is the most common congenital chest wall deformity. It can have a negative effect in exercise tolerance. However, cosmetic features are the most frequent concerns in these patients. The pectus excavatum evaluation questionnaire is a patient-reported outcome (PRO) tool to measure the physical and psychosocial quality of life changes after surgical repair of pectus excavatum. No specific tool has been developed in our languages to evaluate PRO in pectus excavatum patients. Our aim is to translate and culturally adapt the pectus excavatum evaluation questionnaire to European Spanish and Catalan. Methods: Guidelines for translation of PRO were followed. The pectus excavatum evaluation questionnaire, consisting of 34 items, was translated from English to Spanish and to Catalan. Three forward translations and one back translation were performed for each language. Cognitive debriefing interviews were developed. Results: The reconciliation of the forward translations revealed a 14.7% of inconsistencies for each language. The Spanish back translation showed a 64.7% of disagreement with the source, the Catalan 58.8%. Changes in each reconciled version were made to amend the diverting items. 10 patients and their parents participate in the cognitive debriefing for each language, 5 patients had been operated and 5 had not. 4 patients out of 10, for each language, showed difficulties for understanding one of the pectus excavatum evaluation questionnaire items, thus also resulted in a modification of the reconciled version. Conclusion: The translation and cultural adaptation process resulted in the development of a European Spanish and a Catalan version of the pectus excavatum evaluation questionnaire for application in Spanish and Catalan pectus excavatum patients.
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Background Life-threatening arrhythmias have been reported in patients with severe pectus excavatum in absence of other cardiac abnormalities. Literature is scarce regarding diagnosis, cause and management of this problem, particularly regarding the question as to whether the placement of an implantable cardioverter-defibrillator (ICD) is necessary. Case summary A 19-year-old male patient with severe pectus excavatum was scheduled for elective surgical correction. During forward bending for epidural catheter placement, syncope and ventricular fibrillation (VF) occurred resulting in cardiac arrest. After successful cardiopulmonary resuscitation, extensive analysis was performed and showed no cause for VF other than cardiac compression (particularly of the left atrium, right atrium, and ventricle to a lesser degree) due to severe pectus excavatum. Postponed correction by modified Ravitch was performed without ICD placement, with an uneventful post-operative recovery. Eighteen months after surgery, the patient remains well. Upon specific request, he did remember dizzy spells when tying shoelaces. He always considered this unremarkable. Discussion In severe pectus excavatum with cardiac compression, forward bending can decrease central venous return and cardiac output, causing hypotension, arrhythmia, and cardiac arrest. In absence of structural or electric abnormalities, cardiac compression by severe pectus excavatum was considered a reversible cause of VF and ICD placement unnecessary. Patients with cardiac compression due to severe pectus excavatum may report pre-existing postural symptoms upon specific request. When these postural symptoms are present, extreme and prolonged forward bending postures should be avoided.
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A 57-year-old man was referred to our department because of progressive shortness of breath and emaciation. He had experienced pneumothorax three times in the past five years. The patient radiologically showed mild upper-lobe predominant airspace consolidation and severe platythorax and was clinically diagnosed with idiopathic pleuroparenchymal fibroelastosis (PPFE). Although the wedge-shaped shadows in the bilateral lung apexes did not significantly progress, his platythorax gradually worsened during the clinical course. He ultimately died of chronic respiratory failure 1.2 years after the diagnosis. This case demonstrates a rare variant of idiopathic PPFE with progressive platythorax disproportionate to the extent of upper-lobe fibroelastosis.
Objectives: To compare a standard protocol using chest computed tomography (CT) to a non-irradiant protocol involving a low-cost portable 3D scanner and magnetic resonance imaging (MRI) for all pectus deformities based on the Haller index (HI). Methods: From April 2019 to March 2020, all children treated for pectus excavatum or carinatum at our institution were evaluated by chest CT, 3D scanning (iPad with Structure Sensor and Captevia-Rodin4D) and MRI. The main objectives were to compare the HI determined by CT or MRI to a derived index evaluated with 3D scanning, the external Haller index (EHI). The secondary objectives were to assess the inter-rater variability and the concordance between CT and MRI for the HI and the correction index. Results: Eleven patients were evaluated. We identified a strong correlation between the HI with MRI and the EHI (Pearson correlation coefficient = 0.900; P < 0.001), with a strong concordance between a radiologist and a non-radiologist using intra-class correlation for the HI with MRI (intra-class correlation coefficient = 0.995; [0.983; 0.999]) and the EHI (intra-class correlation coefficient = 0.978; [0.823; 0.995]). We also identified a marked correlation between the HI with CT and the EHI (Pearson coefficient = 0.855; P = 0.002), with a strong inter-rater concordance (intra-class correlation coefficient = 0.975; [0.901; 0.993]), a reliable concordance between CT and MRI for the HI and the correction index (Pearson coefficient = 0.886; P = 0.033). Conclusions: Non-irradiant pectus deformity assessment is possible in clinical practice, replacing CT with MRI and 3D scanning as a possible readily-accessible monitoring tool.
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