Sternal cleft and pectus excavatum: an overlooked congenital association?

BACKGROUND: Sternal cleft (SC), a rare thoracic malformation, is associated with pectus excavatum (PE) in 2.6-5% of cases. It remains unclear if these conditions are congenitally linked or if SC repair triggers PE. To investigate the potential higher frequency of PE in SC cases, we conducted a retrospective study of our SC patients. METHODS: We assessed PE incidence, progression, and management in SC patients treated at our institute from 2006 to 2022. When available, we collected pre-SC repair CT scan data, calculating the Haller Index (HI) and Correction Index (CI) and compared them to a selected control group. RESULTS: Among 8 SC patients, 7 had concomitant PE (87.5%), varying in severity. PE management ranged from observation to thoracoplasty, depending on its degree. We observed a significant pre-operative CI difference between SC and control group patients (p < 0.00001). In the last two SC repair cases, we attempted concurrent PE prevention or treatment. CONCLUSION: Our findings suggest an underestimated association between PE and SC in the existing literature. SC patients may exhibit a predisposition to PE from birth, which may become more apparent with growth after SC repair. Consequently, PE prevention or treatment should be considered during SC repair procedures.

Trifid and ventrally curved xiphoid process with two sternal foramina.

In this report, we present the case of a 56-year-old man with an asymptomatic, ventrally curved, trifid xiphoid process with two foramina found incidentally during a CT screening. The objective of this report is to advance the knowledge and recognition of such variations and to discuss the clinical relevance. The xiphoid process was trifid with two xiphoid foramina, one above the other, between the middle and right processes. The right process was longest (approximately 4 cm) and the middle process, the shortest (approximately 2 cm). The upper xiphoid foramen was larger (approximately 0.5 cm) than the lower one (approximately 0.3 cm). No additional musculoskeletal variations were observed on imaging. In summary, this constellation of distinctive variations of the xiphoid process in a single individual provide an opportunity to further discuss the development of the sternum, associated anatomical variations, and potential clinical ramifications from such variations in hopes of advancing knowledge and recognition of such anomalies for clinicians.

Reconstruction of Congenital Sternal Cleft: A Systematic Review of the Literature.

INTRODUCTION: Sternal cleft (SC) is a rare congenital deformity that results from failure of sternal bar fusion. Sternal cleft can be categorized as superior partial, inferior partial, or complete. Each form of SC can present as an isolated defect or in association with other congenital deformities, which presents a unique challenge for reconstructive surgeons. In our systematic review, we aim to summarize the published experience on repair of SCs and present a pragmatic approach to help guide reconstructive planning. METHODS: A systematic review was performed to identify all reported SC cases in literature that underwent sternal reconstruction. RESULTS: Seventy-one studies were identified from 1970 to 2019, which included a total of 115 patients. Superior partial SC was the most common SC variant, accounting for 65.2% (75/115) of all reported cases. There were 31 cases of complete SC (27.0%) and 9 cases (7.8%) of inferior partial SC; 49.6% of the patients (57/115) in our review had isolated SC without any other congenital deformities. Sixty-seven patients (60.3%) were treated with primary closure, with or without secondary maneuvers, such as chondrotomies, cartilage resection, or periosteal flaps. Alternative methods included interposition grafts, with autologous rib graft in 18 patients (15.8%), permanent mesh in 8 patients (7.0%), acellular dermal matrix in 5 patients (4.4%), sternal plate flap in 5 patients (4.4%), and nonthoracic autologous bone grafts in 4 patients (3.5%). CONCLUSIONS: Our review supports that primary closure should be attempted regardless of patient age. For wider sternal gap, reconstruction with an autologous local graft or flap should be considered. When the patient does not have sufficient autologous tissue for a successful sternal reconstruction, alloplastic or allograft interposition options are a reasonable choice.

Sternocleidohyoid muscle: an unreported variant of cleidohyoid muscle.

Sternohyoid, sternothyroid, omohyoid, and thyrohyoid muscles are collectively known as infrahyoid muscles. These muscles frequently show variations in their attachments. Here, an extremely rare variant muscle belonging to this group has been presented. During cadaveric dissection for undergraduate medical students, an additional muscle was found between sternohyoid and superior belly of omohyoid muscles bilaterally in a male cadaver aged approximately 70 years. This muscle took its origin from posterior surface of the manubrium sterni, capsule of the sternoclavicular joint and the posterior surface of the medial part of the clavicle. It was inserted to the hyoid bone between the attachments of sternohyoid and superior belly of omohyoid muscles and was supplied by a branch of ansa cervicalis profunda. There is no report on such a muscle in the literature and it could be named as "sternocleidohyoid muscle". Knowledge of this muscle could be useful in neck surgeries.

EVEN-PLUS syndrome: A case report with novel variants in HSPA9 and evidence of HSPA9 gene dysfunction.

EVEN-PLUS syndrome is a rare condition characterized by its involvement of the Epiphyses, Vertebrae, Ears, and Nose, PLUS other associated findings. We report here the fifth case of EVEN-PLUS syndrome with novel variants c.818 T > G (p.L273X) and c.955C > T (p.L319F) in the HSPA9 gene identified through whole-exome sequencing. The patient is the first male known to be affected and presented with additional features not previously described with EVEN-PLUS syndrome. These features include agenesis of the septum pellucidum, a short chest and sternum, 13 pairs of ribs, a single hemivertebra, laterally displaced nipples, hydronephrosis, unilateral cryptorchidism, unilateral single palmar crease, bilateral clubfoot, and hypotonia. qPCR analysis provides supporting evidence for a nonsense-mediated decay mechanism for the HSPA9 truncating variant. In silico 3D modeling supports the pathogenicity of the c.955C > T (p.L319F) missense variant. The study presented here further describes the syndrome and broadens its mutational and phenotypic spectrum. Our study also lends support to HSPA9 variants as the underlying etiology of EVEN-PLUS syndrome and ultimately provides a better understanding of the molecular basis of the condition.

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.

Honeycomb sterna: an unusual case of a developmental abnormality in the sternum.

This report details an unusual case of a human sternal developmental abnormality of an anatomical specimen part of the skeletal collection curated by University College London, Anthropology Department skeletal collection. This rarely reported developmental abnormality is caused by the non-fusion of lateral ossification centres in the sternebrae, resulting in the mesosternum having a honeycomb-like appearance. Sternal defects are typically underreported in the clinical literature as many cases being asymptomatic that they are typically diagnosed incidentally, as such there is a dearth in our current understanding of the development and anatomical variants of the sternum. Although in recent years, large-scale CT studies have investigated the prevalence of sternal developmental abnormalities, these studies have not reported sternal defects similar to the individual presented in this report. While most sternal defects are clinically uneventful, the lack of awareness of these variants can result in misinterpretation of radiological and pathological findings as such an understanding of anatomical variants even when asymptomatic is vital.

Variation of the sternocleidomastoid muscle: a case report of three heads and an accessory head.

PURPOSE: Anatomical variations of the sternocleidomastoid muscle (SCM) have been observed to occupy multiple origins and insertion points and have supernumerary heads, sometimes varying in thickness. METHODS: An 85-year-old Korean male cadaver was dissected and a SMC with unilateral supernumerary heads was identified. RESULTS: Three heads and an accessory belly of the sternal head were recorded on the left side. There were two clavicular heads, one lateral and one medial. The sternal head originated inferior to the manubrium of the sternum. A small belly arose from the sternal head and merged to the lateral clavicular head on the C5 vertebrae level. These bellies inserted into the mastoid process of the temporal bone. CONCLUSIONS: These variants may be problematic during surgical approaches to the upper neck and occipital, and should thus be appreciated by the clinician.

Topographic evaluation of sternal foramen patients with thoracic computed tomography.

PURPOSE: In our study, we aimed to determine the topographic analysis of sternal foramen cases incidentally detected in patients underwent thoracic computed tomography. MATERIALS AND METHODS: Patients aged 18 and over who were admitted to the thoracic surgery outpatient clinic for various reasons and underwent thoracic computed tomography (CT) between January 1, 2018 and January 1, 2019 were evaluated retrospectively. Thoracic CT scans of all patients with sternal foramen were evaluated by applying 3D bone configuration to evaluate foramina in the sternum and ribs. The data obtained were analyzed statistically by SPSS (Statistical Package for Social Sciences Version 21.0). RESULTS: Nine hundred and twelve patients were evaluated and sternal foramen prevalence was found to be 8.44% in our study. Of the 68 patients included in the study, 48 were male and 20 were female. The sternal foramen was localized in the corpus in 66.2% of the patients, whereas it was localized in xiphoid in 33.8%. Nine patients had scoliosis deformity and three patients had foramen in the rib. CONCLUSION: Sternal foramen is a more common defect than thought, and should be kept in mind in clinical practice to prevent complications.

Sternal cleft and segmental facial hemangiomas in children with phaces syndrome: effectiveness and safety of conservative management and surgical correction.

OBJECTIVE: The aim is to determine the risk factors of sternal cleft and segmental facial hemangiomas association in children with PHACES syndrome. PATIENTS AND METHODS: Materials and methods: 32 inpatient children with segmental facial hemangiomas and 19 children with sternal cleft were investigated concerning the Metry criteria of PHACES syndrome. RESULTS: Results: In 6 children PHACE syndrome was diagnosed. Patients with bilateral S3 hemangiomas (50%, 3/6) had airway involvement with respiratory disorders. Conservative treatment was propranolol monotherapy (66.7%, 4/6), or combination of prednisolone and propranolol (33.3%, 2/6). Duration of propranolol treatment in children with PHACES syndrome was on an average 24.25 ± 4.49 months exceeding the duration of propranolol therapy in children with isolated soft tissue lesions (p<0.05). Primary surgical treatment of sternal cleft performed in children aged 2 (n=3) and 4 (n=1) months. The later period of surgery associated with the localization of hemangioma in the surgery region. Primary repair of sternal cleft was completed successfully in all cases; partial resection of the thymus made closure easier. CONCLUSION: Conclusions: Primary surgical correction of a sternal cleft performed in young children provides good results. Partial resection of the thymus prevents respiratory and cardiovascular complications. Preoperative propranolol treatment averts the hemorrhagic complications in children with hemangiomas in surgical region.

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.

Spontaneous Mediastinal Abscess and Sternal Cleft in the Medically Complex Neonate.

Sternal cleft (SC) is a rare congenital anomaly, occurring with associated developmental anomalies or in isolation. Surgery to reconstruct the sternum is indicated to protect the visceral organs from trauma, to ensure healthy cardiopulmonary function and growth, and to reconstruct the anterior chest wall. Although infection recognized as a postoperative complication following chest wall reconstruction, spontaneous mediastinal infection is rare. To the authors' knowledge, there is only 1 reported case of spontaneous mediastinal infection with concomitant SC in the literature. Here, the authors present a unique case of a medically complicated infant with a SC who presented with a spontaneous mediastinal abscess.

Evaluation of the morphological characteristic and sex differences of sternum by multi-detector computed tomography.

BACKGROUND: Sternum is one of the skeleton parts which have frequently congenital anomalies and variations are commonly used by researchers in determining sex. We evaluated the morphological characteristics and sex-related changes of the sternum in adult individuals using multidetector computed tomography in our study. MATERIALS AND METHODS: Two hundred adults (103 female and 97 male) aged between 18 and 87 years were evaluated. Utilising the morphological characteristics of the sternum based on the multislice images; length, width and the thickness of manubrium, length, width and the thickness of corpus sterni, total length of sternum, sternal angle, sternal index (SI), length of the xiphoid process, the thickness of xiphoid process, the number of indents of xiphoid process were measured and a total of 20 parameters were evaluated by adding age, height and weight to these variables. RESULTS: The mean length of the manubrium, the length of corpus sterni, the length of total sternum, SI, sternal angle were found in females 46.7 ± 5.1, 86.6 ± 9.7, 133.1 ± 1.1, 54.47 ± 10.0 and 163.75 ± 5.79; in males 51.2 ± 6,102.4 ± 13.3, 154.1 ± 13.1, 50.11 ± 10.02 and 162.21 ± 6.17, respectively. We found that Hyrtl's Law and SI did not provide adequate accuracy for sex determination in our patients. It has been detected that the length of the manubrium alone is not helpful for individual samples. Total length of the sternum was found to be more reliable than the length of the manubrium and the length of corpus sterni. We determined sternal cleft and sternal foramen as 0.5% and 3.5%, respectively. CONCLUSIONS: We suggest that the morphometric standards cannot be universally applied and can demonstrate individual differences. The standard rules must be implemented for every population.

[Application of multi-functional modularized prosthesis in reconstruction for sternal defect].

Objective: To investigate the feasibility and effectiveness of sternal reconstruction using a multi-functional modularized sternal reconstruction system. Methods: In June 2016 and August 2017, the modularized sternal prosthesis system was used on two patients for sternal reconstruction in the Department of Thoracic Surgery of Shanghai Pulmonary Hospital, Tongji University. Both patients were female aging 48 and 72 years, respectively, with the primary diagnosis of tumor of the lower sternal body and huge mediastinal tumor. Partial sternal resection and reconstruction was performed through median sternotomy. The multi-functional modularized sternal reconstruction system consisted of manubium, superior sternal body, inferior sternal body, rib and clavicle modules. Each module was designed into 3 to 6 sizes. Appropriate modules were chosen in each case to be assembled as a sternal reconstruction prosthesis. Results: Both operation were smooth, with operation time of 240 minutes and 280 minutes, intraoperative blood loss of 100 ml and 400 ml. The patients were followed up for 18 months and 4 months, respectively. In both cases, the sternal reconstruction was satisfactorily healed, without local infection, fluid accumulation, loose part or dislocation. No local recurrence or distant metastasis was found. Conclusion: The multi-functional modularized sternal reconstruction system can be safely and effectively applied for sternal reconstruction in 2 cases.

[Surgical treatment of pectus carinatum in children]. / Khirurgicheskoe lechenie kilevidnoi deformatsii grudnoi kletki u detei.

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

Sternal Development and Variations and Anomalies in Patients With Microtia: Evaluation Using 3-Dimensional Computed Tomography.

OBJECTIVE: The objectives of this study were to evaluate sternal development and variations in patients with microtia and to identify the incidence of congenital sternal anomalies and then to investigate the interaction between microtia and sternal anomalies. METHODS: A total of 212 consecutive patients received a preoperative 3-dimensional chest computed tomography. A retrospective study was performed with the clinical and imaging data from November 2014 to July 2015. Descriptive statistics, analysis of variance, Spearman analysis, χ test, and Fisher χ test were performed for statistics analysis. RESULTS: We evaluated the ossification centers and developmental variations in the manubrium and body, as well as the xiphoid process, manubriosternal and sternoxiphoidal fusion, and sternal anomalies. Significant variations were observed from person to person. Sternal foramen was detected in 6 male patients (2.8%). All foramina were located in the inferior part of the body. Sternal cleft was observed in 4 cases (1.9%), of which 2 were accompanied by a foramen in the distal part of the sternum. CONCLUSIONS: The development of the different components of the sternum is a process with wide variation among patients with microtia. A different distribution of mesosternal types I to II among our population age range was found, and the incidence of sternal foramina was lower in patients with microtia.

Symptoms in Pectus Deformities: A Scoring System for Subjective Physical Complaints.

Background The literature is silent on the relationship between symptoms and the Haller index. Nor is there a classification of the severity of the physical complaints. Materials and Methods Retrospectively, data from 128 patients (102 funnel, 25 pigeon chest patients, and 1 mixed type) were evaluated. To objectify the symptoms, we developed a score to describe the level of physical ailments. This score includes 10 different symptoms as well as the situation or frequency in which they occur and an impact factor. This depends on how much they affect everyday life. Results Pectus excavatum patients express physical complaints more frequently than pectus carinatum patients who actually suffer more from psychological stress. We could not find a correlation between the Haller index and symptoms or levels of ailment. Conclusion Pectus deformities are likely to cause physical and psychological complaints. Since the subjective symptoms did not show any correlation to the chest severity index, they are supposed to be independent from the deformity's extent.

Sternal malformations and anesthetic management.

Shamberger and Welch classify sternal malformations into four types: thoracic ectopia cordis, cervical ectopia cordis, thoraco-abdominal ectopia cordis, and cleft sternum. Cleft sternum is the most common subset, with a reported incidence of 1 in 50,000 to 100,000 live births, representing 0.15% of all anterior chest wall malformations. Acostello et al further classify cleft sternum into complete or partial (superior, medium, inferior) with a simple superior partial cleft sternum being by far the most common with an orthotopic heart, intact pericardium, and normal skin coverage. Associated anomalies with superior partial cleft sternum are rare, but can include cervicofacial hemangiomas, midline raphe from the tip of the cleft to the umbilicus, and PHACES (posterior fossa malformations, facial hemangiomas, arterial anomalies with coarctation of aorta, cardiac defects, eye abnormalities, sternal cleft, and supraumbilical raphe) syndrome. The more rare inferior partial clefts are associated with thoraco-abdominal ectopia cordis as part of the Pentalogy of Cantrell (omphalocele, anterior diaphragmatic hernia, sternal cleft, ectopia cordis, ventricular septal defect/left ventricular diverticulum). This review summarizes the current knowledge of all four types of sternal malformations, and provides guidance for optimal anesthetic and perioperative care of these children.

Morphological approach of the sternal foramen: an anatomic study and a short review of the literature.

BACKGROUND: The sternal foramen (SF) constitutes a specific anatomic defect in sternum, indicating an impaired fusion of ossificated segments, which occurs either in an anatomical part of the sternum or in sternal joints. The aim of this article is to provide baseline statistical data about the variations of the SF, to present a short review of the relevant literature and to compare results with other studies and populations. MATERIALS AND METHODS: We review relevant literature, and we present data obtai-ned from skeletal samples of known population and sex. A total of 35 well-preserved dried sterna from the prefecture of Eastern Macedonia and Thrace, Greece, were selected: 20 men and 15 women with a mean age of 55 ± 6 years old. Measurements were made with a sliding calliper and photographic documentation. RESULTS: The incidence of the SF in the 35 dried specimens was 14.2%, 4 men (20% of male sample) and 1 woman (6.6% of female sample) and 80% of sternal foramina were observed in male individuals. The SF was found in the sternum body (2 cases, 40% of foramina), in xiphoid process (2 cases, 40% of foramina) and in sternoxiphoidal junction (1 case, 20% of foramina). All of the sterna presented 1 single visible SF. Two anatomically unique cases were identified throughout these 5 sterna, both belonging in male subjects. CONCLUSIONS: The SF constitutes a relatively common variation with great radiological, clinical, and forensic significance. Presence of a SF with irregular bony margins complicates considerably radiological differential diagnosis. Awareness of this important anatomic variation is fundamental for clinicians and autopsy pathologists, in order to avoid severe fatal complications and elucidate the exact cause of death, respectively.