Pectus Arcuatum: A Pectus Unlike Any Other.

BACKGROUND: Pectus arcuatum is often mistaken for a type of pectus carinatum. However, pectus arcuatum is a unique clinical form of pectus caused by premature obliteration of the sternal sutures (manubrial sternum, four sternebrae and xiphoïd process), whereas pectus carinatum is due to abnormal growth of the costal cartilage. In order to better describe pectus arcuatum, we analysed the files of patients with pectus arcuatum followed in our centers. METHODS: Multicenter retrospective study of young patients' files diagnosed with pectus arcuatum. RESULTS: The clinical diagnosis of pectus arcuatum was made in 34 patients with a mean age at diagnosis of 10.3 years (4-23 years). A chest profile X-ray or a CT scan was performed in 16 patients (47%) and confirmed the diagnosis of PA by the presence of a sternal fusion. It was complete in 12 patients. A malformation was associated in 35% of cases (Noonan syndrome 33%, scoliosis 25% or cardiopathy 16%). 11 patients (32%) had a family history of skeletal malformation. Orthopedic treatment was initiated in 3 patients without any success. 11 patients underwent surgical correction, which was completed in 7 of them. CONCLUSION: The diagnosis of pectus arcuatum is based on clinical experience and if necessary, on a profile chest X-ray showing the fusion of the sternal pieces. It implies the search for any associated malformations (musculoskeletal, cardiac, syndromic). Bracing treatment is useless for pectus arcuatum. Corrective surgery, based on a sternotomy associated with a partial chondro-costal resection, can be performed at the end of growth. LEVEL OF EVIDENCE: IV.

Combined surgical correction of pectus carinatum and juvenile kyphosis.

Pectus carinatum may rarely be associated with kyphosis. However, the correlation between both conditions is not well reported. Therefore, there are no reports for combined correction of both deformities in the same patient. Moreover, studies estimating the kyphosis prevalence in patients with pectus carinatum are lacking. To our knowledge, this is the first paper to present such a case. We report an 18-year-old boy with both pectus carinatum and kyphosis that were surgically corrected in a combined procedure. The indication of surgery is cosmetic, and the postoperative recovery included pneumothorax but was otherwise uneventful and satisfactory.

Orthotic Bracing or Minimally Invasive Surgery? A Summary of 767 Pectus Carinatum Cases for 9 Years.

Orthotic bracing and minimally invasive surgery are currently the treatment methods for pectus carinatum. We present our experience with the advantages, method selection criteria, and precautions for both treatment methods. A total of 767 pediatric patients (596 boys and 171 girls) with pectus carinatum were retrospectively analyzed. All of them received orthotic bracing, and 108 pediatric patients received minimally invasive surgery, achieving good outcomes. Among the 767 pediatric patients, 644 obtained satisfactory chest appearance through orthotic bracing, with a success rate of 84.0%. Younger pediatric patients had better orthotic outcomes. Among the 123 failure cases, 108 pediatric patients underwent minimally invasive surgery as the treatment. Seventy-six pediatric patients with simple chondrogladiolar prominence underwent a minimally invasive sternal depression procedure, whereas 32 pediatric patients with complex chondromanubrial prominence underwent surgery. All 108 pediatric patients successfully completed the surgery. The operation time was 112.8 ± 23.5 min, and the average length of hospital stay after the surgery was 7 days. A follow-up was conducted for at least 3 months and up to 3 years. The orthotic effect was satisfactory. For younger pediatric patients with pectus carinatum, noninvasive orthotic bracing treatment should be considered first. For older pediatric patients, the failure rate of the bracing was higher, and the outcomes were often unsatisfactory. Especially for patients over 15 years old, minimally invasive sternal depression may be the preferred treatment for pectus carinatum.

Effectiveness of Compressive External Bracing in Patients with Flexible Pectus Carinatum Deformity: A Review.

There has been a growing interest in the nonoperative treatment of chest wall deformities over the last few decades; with the advent of external compressive orthotics, similar outcomes have been reported compared with surgical intervention. There have been fewer major complications reported with dynamic compression bracing on the chest wall; however, the Achilles heel of this approach still lies with treatment tolerability and compliance. A Medline literature search was undertaken to evaluate the evidence concerning the techniques, modifications, and outcomes associated with external compressive bracing in the setting of pectus carinatum. Sixteen articles were integrated after literature review and data were collected on methods of assessing pectus carinatum (degree of severity as well as type), patient selection protocol (i.e., suitability for external compressive bracing), the bracing protocol itself, duration of treatment, metrics used to assess outcome, and success, compliance, and dropout rate, and length of long-term follow-up. Compressive external bracing appears to be a safe and well tolerated nonsurgical treatment option for young patients with flexible pectus carinatum deformities. However, there is still a need for robust level I randomized data from multiple centers with a clearly standardized bracing protocol, objective measurement of outcomes, and recording of results at the end of the bracing treatment program in sufficiently powered sample sizes over a significant follow-up period.

Patient and healthcare professional satisfaction with 3D imaging new technologies for medical purposes. / Satisfacción de pacientes y profesionales con las nuevas tecnologías de imagen tridimensional aplicadas a la medicina.

BACKGROUND AND OBJECTIVES: Advances in the field of three-dimensional scanning have enabled the development of instruments that can generate images that are useful in medicine. On the other hand, satisfaction studies are becoming increasingly important in the evaluation of quality in healthcare. We aimed to evaluate patients' and professionals' satisfaction with the use of a three-dimensional scanner applied to chest wall malformations. MATERIAL AND METHODS: In the framework of a study to validate the results of three-dimensional scanning technology, we developed questionnaires to measure satisfaction among patients and professionals. We analyzed the results with descriptive statistics. RESULTS: We included 42 patients and 10 professionals. Patients rated the speed and harmlessness positively; the mean overall level of satisfaction was 4.71 on a scale from 1 to 5. Among professionals, the level of satisfaction was lower, especially with regards to the treatment of the image; the mean overall level of satisfaction was 3.1. CONCLUSIONS: Patients rated 3D scanning technology highly, but professionals were less satisfied due to the difficulty of treating the images and lack of familiarity with the system. For this technology to reach its maximum potential, it must be simplified and more widely disseminated.

Prevalence of pectus excavatum (PE), pectus carinatum (PC), tracheal hypoplasia, thoracic spine deformities and lateral heart displacement in thoracic radiographs of screw-tailed brachycephalic dogs.

Pectus excavatum, thoracic spine deformities, tracheal hypoplasia and lateral heart displacement are frequently described in brachycephalic dog breeds. Pectus carinatum is described sporadically, although the authors' observations demonstrate that it may occur in certain brachycephalic dog breeds. It was hypothesised that dogs of screw-tailed brachycephalic breeds carry a greater risk of these anomalies than normal-tailed brachycephalic breeds, and that there could a relation between the presence of pectus excavatum or pectus carinatum and thoracic spine deformities, tracheal hypoplasia and lateral heart displacement. During retrospective studies, these anomalies were identified in lateral and dorso-ventral radiographs of the thorax in brachycephalic dog breeds. A statistical analysis revealed that the frequency of pectus excavatum occurrence in screw-tailed and normal-tailed brachycephalic dog breeds is similar. The greatest risk of pectus excavatum occurrence is carried by two breeds: Maltese (60%) and English Bulldog (58%), while for pectus carinatum: Pug (41%) and French Bulldog (18%). Dogs of screw-tailed brachycephalic breeds carry a greater risk of kyphosis (p < 0.0001), tracheal hypoplasia occurrence (p < 0.0001), compared to "normal-tailed" breeds. The hypothesis concerning a relation between the presence of pectus excavatum or pectus carinatum and the other anomalies studied was not confirmed (p > 0.05). It was demonstrated that in dogs of brachycephalic breeds there was a greater risk of co-incidence between kyphosis of the thoracic spine and lateral heart displacement (p = 0.038), as well as kyphosis of the thoracic spine and tracheal hypoplasia (p = 0.003).

Pectus Carinatum: a non-invasive and objective measurement of severity.

To date, standard methods for assessing the severity of chest wall deformities are mostly linked to X-ray and CT scans. However, the use of radiations limits their use when there is a need to monitor the development of the pathology over time. This is particularly important when dealing with patients suffering from Pectus Carinatum, whose treatment mainly requires the use of corrective braces and a systematic supervision. In recent years, the assessment of severity of chest deformities by means of radiation-free devices became increasingly popular but not yet adopted as standard clinical practice. The present study aims to define an objective measure by defining a severity index (named External Pectus Carinatum Index) used to monitor the course of the disease during treatment. Computed on the optical acquisition of the patients' chest by means of an appositely devised, fast and easy-to-use, body scanner, the proposed index has been validated on a sample composed of a control group and a group of Pectus Carinatum patients. The index proved to be reliable and accurate in the characterization of the pathology, enabling the definition of a threshold that allows to distinguish the cases of patients with PC from those of healthy subjects. Graphical abstract.

A Simplified Method for Three-Dimensional Optical Imaging and Measurement of Patients with Chest Wall Deformities.

BACKGROUND: Pectus excavatum and carinatum are two of the most commonly observed chest wall deformities in pediatrics. The standard diagnostic evaluation for these conditions includes either chest radiograph (CXR) or computed tomography (CT). Our research aims to develop a novel and reliable way of quantifying chest wall deformities in the clinic setting without radiation exposure. METHODS: Using a handheld structured light scanner, we created three-dimensional (3D) models of patients with chest wall deformities through an IRB-approved protocol. Raters from a variety of backgrounds were then asked to take measurements based on the 3D model utilizing commercially available 3D graphical software. The standard deviation of the measurements and intraclass correlation coefficient (ICC) were then calculated to quantify inter-rater reliability. RESULTS: Sixty patients with pectus excavatum (Haller index range 2.0-6.38) and pectus carinatum were enrolled and imaged in our outpatient clinic using a structured light scanner. Five patients were used to verify interuser reliability. The standard deviation of all the measurements was 2.2 mm. The ICC for absolute agreement was 0.99139, with 1.0 being perfect correlation. CONCLUSION: Structured light scanners provide an alternative approach to quantifying chest wall deformities in pediatric patients without radiation exposure. Our method is highly reliable, even among users with minimal image processing or 3D modeling experience. Our protocol can potentially be used to track treatment progress in children with chest wall deformities.

Customized Cutting Template to Assist Sternotomy in Pectus Arcuatum.

PURPOSE: Pectus arcuatum is an anterior chest wall deformity that requires transverse wedge sternotomy. Determining and delivering the correct cutting angle are crucial for successful correction. This report describes the early clinical experience with a novel cutting template technology able to deliver the optimal cutting angle. DESCRIPTION: From patients' computed tomographic scans, the optimal cutting angle is obtained using computer-aided design. A template comprising slots tilted at the right cutting angle and a safety block to avoid damaging the posterior periosteum is printed through additive manufacturing. EVALUATION: The template allows surgeons to perform a precise wedge sternotomy, safely sparing the posterior periosteum in all patients, without complications. Postoperative chest roentgenograms and clinical photographs demonstrate optimal sternal realignment and cosmetic outcome. In this report, the mean operative time was 110 minutes. All patients were successfully discharged, with a mean length of stay of 4 days. CONCLUSIONS: Transverse wedge sternotomy aided by a computer-aided design-devised cutting template may reduce the technical challenge of this procedure, thereby increasing its safety and reducing operative times and hospital stay. Further research on long-term patient outcomes is necessary.

An Evidence-Based Approach to Management of Pectus Excavatum and Carinatum.

Pectus excavatum (PE) and pectus carinatum (PC) are the most common congenital chest wall anomalies. Current research suggests that PE and PC may result from overgrowth of the sternocostal cartilages. This can produce a deformation that displaces the sternum inward as in PE or outward as in PC. The etiology, clinical presentation, evaluation, and management of PE and PC are reviewed. Varied clinical presentations, cardiopulmonary effects, and psychosocial aspects are described.

Molecular analysis provides further evidence that Chitayat syndrome is caused by the recurrent p.(Tyr89Cys) pathogenic variant in the ERF gene.

Chitayat syndrome (CHYTS, MIM #617180) is a rare autosomal dominant clinical condition caused by a single missense pathogenic variant in the ERF gene (19q13.2, MIM*611888), which encodes the ETS2 Repressor Factor (ERF) protein. The characteristic features reported to date for this condition are facial dysmorphism, hyperphalangism and respiratory complications during the newborn period. Herein, we report the sixth patient worldwide with a confirmed molecular diagnosis of CHYTS. Our documentation of pectus carinatum, hypoplastic phalanges (as in two previously described patients), and lack of hyperphalangism broadens the phenotypic spectrum of CHYTS. Moreover, our identification of a heterozygous mutation [c.266A>G or p.(Tyr89Cys)] [rs886041001] in this patient provides further evidence that this condition is caused by a recurrent pathogenic variant in ERF.

Measuring the impact of brace intervention on pediatric pectus carinatum using white light scanning.

BACKGROUND: Evaluation of Pectus Carinatum (PC) deformity in patients undergoing bracing is limited to subjective assessment of the chest through physical exam and photography. White Light Scanning (WLS) is a novel 3D imaging modality and offers an objective alternative that is quick, inexpensive, and safe. We previously demonstrated the feasibility of using a WLS-derived proxy for Haller index, called the Hebal-Malas Index (HMI), in measuring the surgical correction of Pectus Excavatum. The purpose of this study was to demonstrate the use of WLS to measure the severity of pre- and postbracing intervention of PC deformities and assess corrected difference between the two scans. METHODS: We conducted a prospective review of preintervention WLS scans in pediatric patients with PC from 2015 to 2017. HMI was obtained from the preintervention and postintervention WLS scans. Analysis assessed the differences of pre- and postbracing intervention of measurements. RESULTS: Of 32 patients with both pre- and postbracing scans, 13 (34%) showed improvement of more than 10%, 21 (55%) showed slight improvement of 1%-10%, and 4 (11%) did not improve at follow-up. The average postbracing change in the WLS-derived HMI was 0.10 (SD:0.11). The average length of bracing days was 331.4 (SD: 127.3) with an average of 6.8 h worn per day. Compliance was defined as patient reported utilization of the brace. Patients who were compliant showed a significant improvement (p = 0.004) compared to those who were not compliant (Table 2). However, even patients with moderate compliance still improved in many instances. Change in height was a significant factor correlating with improvement. Children who grew more while wearing a brace showed greater improvement in their deformity. CONCLUSION: Using this technique, we have the ability to objectively quantify the impact of bracing on the severity of PC deformity and measure change in deformity over time. TYPE OF STUDY: Prospective study. LEVEL OF EVIDENCE: Level IV.

The use of sternal wedge osteotomy in pectus surgery: when is it necessary?

BACKGROUND: The Ravitch procedure is a well-established surgical procedure for correction of chest wall deformities. Sternal wedge osteotomy is an important part of this procedure. We studied the incidence of wedge osteotomy with respect to the type of chest wall deformity in patients undergoing surgical correction with the use of a recently developed chest wall stabilization system. METHODS: A total of 47 patients, 39 (83%) male and 8 (17%) female with a mean age of 14.9 ± 2.1 years, underwent the Ravitch procedure. Twenty-four (51.1%) had pectus carinatum, 19 (40.4%) had pectus excavatum, and 4 (8.5%) had pectus arcuatum. A conventional or oblique sternal wedge osteotomy was performed as indicated, followed by chest wall stabilization using the MedXpert system. RESULTS: Of the 47 patients, 27 (57.4%) had a sternal wedge osteotomy. All cases of pectus arcuatum and redo cases underwent sternal wedge osteotomy. Pectus excavatum cases tended to have a greater incidence of wedge osteotomy compared to pectus carinatum cases (68.4% vs. 41.7%, p = 0.052). Patients with more resected ribs had a greater rate of wedge osteotomy (63.4%) compared to those with fewer resected ribs (16.7%, p = 0.043). CONCLUSIONS: A sternal wedge osteotomy is more commonly performed in patients with pectus excavatum compared to those with pectus carinatum. All redo and pectus arcuatum cases need a wedge osteotomy for proper correction. Wedge osteotomy is very likely in more aggressive corrections with more rib resections.

Dynamic Compression System: An Effective Nonoperative Treatment for Pectus Carinatum: A Single Center Experience in Basel, Switzerland.

Background Several nonoperative treatments are currently available for the correction of pectus carinatum (PC). Objective The objective of this study is to report our single center experience with the dynamic compression system (DCS). Materials and Methods The DCS is a rigid aluminum brace. PC is reshaped into a normal appearance through anterior-posterior pressure and lateral expansion of the chest. Patients with chondrogladiolar PC were considered suitable for the nonoperative treatment with DCS. Results In this study, 53 of 68 children (78%) with chondrogladiolar PC were assessed retrospectively: 2 children were corrected by surgery, 12/53 (23%) treated by a conventional orthesis, 11/53 (21%) remained without therapy because of minor PC, and 36/53 (68%) were treated using the DCS. Of these 36 patients, 17 (47%) are already cured with a good (7/17) to excellent (10/17) cosmetic result after a median treatment period of 9 months (range, 2.5-16 months). The mean daily time of wearing of the device for those 17 patients was 9 hours (range, 5-18). None abandoned the treatment and there were almost no complications. Conclusions Lateral expansion of the chest and the possibility to measure the applied pressure seemed to be the key to DCSs success. We propose the DCS as first choice in the treatment of chondrogladiolar PC in children.

Presentation of parathyroid adenoma with genu valgum and thoracic deformities.

Parathyroid adenoma is the main cause of primary hyperparathyroidism. It is usually asymptomatic and occurs more commonly in adults. It presents with raised parathormone (PTH) and Ca+ levels in serum. Its presentation in adolescence is rare. We report one such incidence of a 14 years old girl who presented with bone pains short stature, and generalized muscle wasting. She was found to have genu valgum at the knee joint, pectus carniatum, scoliosis and cystic changes in pelvis and calvarium. Biochemical investigations and parathyroid Tc-99mMIBI scan confirmed the diagnosis of a parathyroid adenoma. The gland was removed by parathyroidectomy. Till date 12 such cases are reported and none had thoracic, vertebral or calvarium involvement.

The etiology of pectus carinatum involves overgrowth of costal cartilage and undergrowth of ribs.

PURPOSE: We compared the length of costal cartilage and rib between patients with symmetric pectus carinatum and controls without anterior chest wall protrusion, using a 3-dimensional (3D) computed tomography (CT) to evaluate whether the overgrowth of costal cartilage exists in patients with pectus carinatum. SUBJECTS AND METHODS: Twenty-six patients with symmetric pectus carinatum and matched twenty-six controls without chest wall protrusion were enrolled. We measured the full lengths of the 4th-6th ribs and costal cartilages using 3-D volume rendering CT images and the curved multiplanar reformatted (MPR) techniques. The lengths of ribs and costal cartilages, the summation of rib and costal cartilage lengths, and the costal index [length of cartilage/length of rib * 100 (%)] were compared between the patients group and the control group at 4th-6th levels. RESULTS: The lengths of costal cartilage in patient group were significantly longer than those of control group at 4th, 5th and 6th rib level. The lengths of ribs in patient group were significantly shorter than those of control group at 4th, 5th and 6th rib level. The summations of rib and costal cartilage lengths were not longer in patients group than in control group. The costal indices were significantly larger in patients group than in control groups at 4th, 5th and 6th rib level. CONCLUSION: In patients with symmetric pectus carinatum, the lengths of costal cartilage were longer but the lengths of rib were shorter than those of controls. These findings may supports that the overgrowth of costal cartilage was not the only factor responsible for pectus carinatum.