RESUMEN
Pectus defects are a group of congenital conditions found in approximately 1 in 250 people, where the sternum is depressed back towards the spine (excavatum), protrudes forwards (carinatum) or more rarely is a mixture of both (arcuatum or mixed defects). For the majority of patients, it is well tolerated, but some patients are affected psychologically, physiologically or both. The deformity becomes apparent at a young age due to the growth of the ribs and the cartilage that links them to the sternum. The majority of defects are mild and are well tolerated, i.e. they do not affect activity and do not cause psychological harm. However, some young people develop lower self-esteem and depression, causing them to withdraw from activities (such as swimming, dancing) and from interactions that might 'expose' them (such as sleepovers, dating, going to the beach and wearing fashionable clothes). This psychological harm occurs at a crucial time during their physical and social development. A small number of patients have more extreme depression of their sternum that impedes their physiological reserve, which can occur when engaging in strenuous exercise (such as running) but can also limit moderate activity such as walking and climbing stairs. The effects can be so extreme that symptoms occur at rest or cause life-threatening compression of the major blood vessels and organs. The group of patients with physiological impairment usually also suffer from low self-esteem and depression. This paper summarizes the current evidence for the different treatment strategies for this condition, including supportive care, psychological support and non-surgical techniques including bracing and vacuum bell therapy. We also consider surgical techniques including the Ravitch procedure, the Nuss procedure (minimally invasive repair of pectus excavatum), pectus implants and other rare procedures such as Pectus Up. For the majority of patients, supportive care is sufficient, but for a minority, a combination of the other techniques may be considered. This paper also outlines best practice guidance for the delivery of such therapies, including standardized assessment, consent to treatment, audit, quality assurance and long-term support. All the interventions have risks and benefits that the patient, parents and clinicians need to carefully consider and discuss when deciding on the most appropriate course. We hope this evidence review of 'Best Practice for Pectus' will make a significant contribution to those considerations and help all involved, from patients to national policy makers, to deliver the best possible care.
Asunto(s)
Pectus Carinatum , Humanos , Pectus Carinatum/terapia , Tórax en Embudo/cirugía , Tórax en Embudo/terapia , Esternón/anomalías , ConsensoRESUMEN
This article reviews pectus excavatum, carinatum, and arcuatum. Topics covered include etiology, epidemiology, associated syndromes, physiologic impact, workup, indications for treatment, surgical and nonsurgical therapy, results, complications, and emerging therapies. Pectus excavatum is an inward deformation of the sternum and/or anterior chest wall. Pectus carinatum is ether an outward protrusion or tilt of the sternum with potential psychological impact, but no demonstrated physiologic impact. Nonoperative compression bracing is successful in carinatum patients with chest wall flexibility who are compliant with a bracing program. Pectus arcuatum is an abnormally short, fully fused sternum with a high anterior protrusion.
Asunto(s)
Tórax en Embudo , Pectus Carinatum , Humanos , Tórax en Embudo/terapia , Tórax en Embudo/diagnóstico , Pectus Carinatum/terapia , Pectus Carinatum/diagnóstico , Niño , Tirantes , Esternón/anomalíasRESUMEN
CASE PRESENTATION: A newborn girl presented to the hospital on the first day of life because of respiratory failure. She was born at home at 37 weeks' gestation with minimal prenatal care and was found to be small for gestational age. The patient was found to have partial sternal agenesis and sternal cleft, cutis aplasia, left facial hemangioma, micrognathia, wide-spaced nipples, and low-set ears. The mother's and baby's urine toxicology screening were positive for amphetamines. Chest radiographs on admission showed bilateral hazy opacities. CT scan of the chest showed an absent sternum with midline chest wall concavity. The patient was monitored preoperatively in the cardiac ICU for risks of arrythmia, respiratory failure, altered cardiac output, and acute cardiopulmonary decompensation.
Asunto(s)
Esternón , Humanos , Femenino , Esternón/anomalías , Esternón/diagnóstico por imagen , Recién Nacido , Anomalías Múltiples/diagnóstico , Tomografía Computarizada por Rayos X , Hemangioma/diagnóstico , Hemangioma/complicaciones , Hemangioma/diagnóstico por imagen , Anomalías Musculoesqueléticas/diagnóstico por imagen , Anomalías Musculoesqueléticas/diagnósticoRESUMEN
INTRODUCTION: Minimally invasive repair of pectus carinatum (MIRPC) has been performed using the Abramson technique in which the bar that compresses the sternum is fixed with steel wires on the ribs. A 14-year-old patient underwent to a MIRPC using a sandwich technique in which two metallic bars fixed with bridges were implanted below the sternum under thoracoscopic vision, and another bar in a subcutaneous tunnel was implanted above. This technique has the potential to avoid specific problems related to the original technique like loosening of support for correction (broken wire), avoidance of induction of pectus excavatum or subcutaneous tissue adhesion.
Asunto(s)
Pectus Carinatum , Humanos , Pectus Carinatum/cirugía , Adolescente , Masculino , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Procedimientos Quirúrgicos Mínimamente Invasivos/instrumentación , Toracoscopía/métodos , Toracoscopía/instrumentación , Esternón/cirugía , Esternón/anomalías , Hilos Ortopédicos , Resultado del TratamientoRESUMEN
BACKGROUND: Pectus arcuatum, also known as horns of steer anomaly or Currarino-Silverman Syndrome, is a distinct chest wall anomaly characterized by severe manubriosternal angulation, a shortened sternum, and mild pectus excavatum. The anomaly is typically repaired using open techniques, employing orthopedic fixation devices. Here, we report the results of a minimally invasive hybrid procedure to repair pectus arcuatum. METHODS: The procedure combines a standard Nuss procedure to correct the depressed sternum with a short upper chest (in boys) or inter-mammary (in girls) incision for bilateral subperichondrial resection of the upper costal cartilages, osteotomy, and correction of the manubrial angulation. The medical records of all patients who underwent the procedure over the last 10 years were reviewed. RESULTS: Five patients, 3 boys and 2 girls, aged 14 to 17 years, underwent the procedure. Three patients had their pectus bars removed 3-4 years after repair. Follow-up after correction ranged from 6 months to 7 years. Good correction resulted in all patients achieving recovery without complications and recurrence. To date, all patients have been satisfied with their results. CONCLUSIONS: The minimally invasive hybrid procedure adequately corrects pectus arcuatum with minimal scarring and high satisfaction.
Asunto(s)
Tórax en Embudo , Procedimientos Quirúrgicos Mínimamente Invasivos , Osteotomía , Humanos , Masculino , Femenino , Adolescente , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Tórax en Embudo/cirugía , Osteotomía/métodos , Esternón/cirugía , Esternón/anomalías , Resultado del Tratamiento , Pared Torácica/cirugía , Pared Torácica/anomalías , Estudios Retrospectivos , Estudios de SeguimientoRESUMEN
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.
Asunto(s)
Tórax en Embudo , Anomalías Musculoesqueléticas , Esternón/anomalías , Humanos , Tórax en Embudo/complicaciones , Tórax en Embudo/diagnóstico por imagen , Tórax en Embudo/epidemiología , Estudios Retrospectivos , Anomalías Musculoesqueléticas/diagnóstico por imagen , Anomalías Musculoesqueléticas/epidemiología , Anomalías Musculoesqueléticas/cirugía , GenotipoAsunto(s)
Neoplasias Óseas , Esternón , Humanos , Esternón/anomalías , Esternón/diagnóstico por imagen , Lactante , Neoplasias Óseas/diagnóstico , Masculino , FemeninoRESUMEN
OBJECTIVE: The present case study aims at drawing attention to a very rare presentation of the sternalis muscle noticed during routine dissection, and is intended to highlight the clinical significance and usefulness of this unique muscle in reconstructive surgeries, especially of the breast. CASE REPORT: Though many morphological variants of the muscle have been reported, we came across a unique bilateral sternalis muscle during routine dissection for undergraduate medical teaching, in an 80-year-old male cadaver. The muscle originates on both sides from the external oblique aponeurosis from the fleshy belly, and after becom-ing tendinous, converges in the midline to form a common tendon at the level of the sternal angle, and then splits again into two tendons which become continuous with the ipsilateral sternocleidomastoid. CONCLUSION: Notwithstanding the fact that the presence of a sternalis can be misdiagnosed as a wide range of anterior chest wall lesions and tumors, especially with misdiag-nosis of breast masses in routine mammograms, it has great use as a muscular flap for reconstructive surgeries of the anterior chest wall, head, neck and breast.
Asunto(s)
Cadáver , Músculo Esquelético , Humanos , Anciano de 80 o más Años , Masculino , Pared Torácica , Esternón/anomalías , DisecciónRESUMEN
INTRODUCTION: Traditional surgical methods have been difficult for patients with chest wall deformities, but the use of the Electrical Sternum Lifting System (ESLS) has made the surgery easier. MATERIALS AND SURGICAL TECHNIQUE: Patients with a sternum-to-vertebral distance of less than 80 mm on preoperative computed tomography (CT) scan routinely underwent sternal lift using the ESLS. The ESLS was effective in securing the operative field while suspending the sternum, allowing adequate observation of the left atrium, left ventricle, and the mitral valve, and safe mitral valve plasty. The use of the lifting device did not interfere with the robot arms, and the space between the sternum and vertebrae was widened with only a 3 mm wound to move the mitral valve surface in the sagittal plane, making the repair easy and accurate under robotic assistance. The effort to attach the ESLS was not difficult, and the postoperative cosmetic outcomes were excellent. Sixty-three out of 1002 patients (6.3%) underwent sternal elevation using ESLS. There were 19 males and 44 females with a mean age of 50.9 ± 14.0 years. The average of sternum-to-vertebral (S/V) distance was 72.4 ± 8.9 cm. Two patients had S/V distance of more than 80 mm but ESLS was used because of scoliosis.
Asunto(s)
Tórax en Embudo , Procedimientos Quirúrgicos Robotizados , Robótica , Masculino , Femenino , Humanos , Adulto , Persona de Mediana Edad , Tórax en Embudo/cirugía , Depresión , Elevación , Resultado del Tratamiento , Esternón/cirugía , Esternón/anomalíasRESUMEN
We describe reconstruction of a sternal cleft in a neonate using acellular dermal matrix, full-thickness calvarial bone graft and myocutaneous pectoralis flaps.
Asunto(s)
Dermis Acelular , Anomalías Musculoesqueléticas , Esternón , Humanos , Recién Nacido , Esternón/anomalíasRESUMEN
Sternal cleft accompanied by pectus excavatum is a rare type of congenital anomaly of the chest wall. Surgical correction is a suitable approach to restore the heart, large vessels, and respiratory dynamics early. This is a report of the successful surgical correction of upper sternal cleft anomaly accompanied by pectus excavatum in a child. The pectus excavatum was corrected without the use of any prosthesis. The cleft was closed by primary approximation with enough dissected pectoralis major muscle and partial thymectomy, mobility, and flexibility ensured by pectus correction. The integrity of the sternum and the chest wall was normal at the end of the 12-month follow-up period.
Asunto(s)
Tórax en Embudo , Anomalías Musculoesqueléticas , Niño , Humanos , Tórax en Embudo/diagnóstico , Tórax en Embudo/diagnóstico por imagen , Esternón/diagnóstico por imagen , Esternón/cirugía , Esternón/anomalías , Anomalías Musculoesqueléticas/diagnóstico , Anomalías Musculoesqueléticas/diagnóstico por imagen , CorazónRESUMEN
BACKGROUND Sternal cleft is a greatly rare congenital thoracic deformity, arising from a failure of the sternal bars fusion process that should be completed in the fetal period, the incidence of which is less than 0.15%. CASE REPORT Herein, we present a case report of a newborn girl having a superior congenital sternal cleft. After the baby was born, scar-like tissue was found in the middle of the chest and extended to the root of the umbilical cord. Based on the imaging data, this newborn was diagnosed with sternal cleft belonging to the superior sternal cleft and not associated with other congenital deformities. CONCLUSIONS As a rare congenital thoracic deformity, postpartum diagnosis of the sternal cleft mainly is currently based on medical imaging, including thoracic computed tomography (CT), three-dimensional (3D) reconstruction CT, and magnetic resonance imaging (MRI). Sternum cleft not only affects the aesthetic appearance but also leads to the destruction of the bone structure of the thorax, resulting in opposing thoracic movements. Therefore, early diagnosis and early treatment play significant roles in the treatment of this congenital sternal deformity. Regardless of whether there are clinical symptoms of sternal cleft, primary repair surgery must be done as soon as possible and during the neonatal period is best, in which simple surgical techniques achieve remarkable effects.
Asunto(s)
Anomalías Musculoesqueléticas , Esternón , Recién Nacido , Femenino , Humanos , Esternón/diagnóstico por imagen , Esternón/cirugía , Esternón/anomalías , Anomalías Musculoesqueléticas/diagnóstico por imagen , Anomalías Musculoesqueléticas/cirugía , Tomografía Computarizada por Rayos X , RadiografíaRESUMEN
Sternal cleft is a rare malformation of the midline fusion of the sternal bars; the most common form is the superior partial defect. Surgical correction with primary closure is the gold standard. It is recommended that the procedure be performed before 3 months of age because of the greater compliance and maximal flexibility of the thoracic wall. These features ensure a safer repair with a low risk of complications and allow for a less extensive procedure that does not require the use of additional techniques. A midline incision is performed in the anterior thoracic wall, and the major pectoralis flaps are raised. The main surgical goal is to change the remaining sternum from a U to a V shape. Transfixing interrupted sutures are placed in the cartilaginous borders for midline closure. Hemodynamics and ventilation are monitored at this time. Closure is performed by layers.
Asunto(s)
Anomalías Musculoesqueléticas , Pared Torácica , Humanos , Recién Nacido , Anomalías Musculoesqueléticas/cirugía , Esternón/anomalías , Esternón/cirugía , Colgajos Quirúrgicos , Pared Torácica/cirugíaRESUMEN
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.
Asunto(s)
Esternón , Apófisis Xifoides , Humanos , Masculino , Persona de Mediana Edad , Esternón/anomalías , Esternón/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
Abnormalities of the sternal and peri-sternal regions are commonly seen in clinical practice and may be one of the important causes of chest pain particularly anterior chest wall pain. While reading computed tomography (CT) of the chest for evaluation of chest pain, the sternal region is either easily overlooked or its abnormality is often detected incidentally. This article will provide an overview of normal sternal anatomy and congenital variants as well as a variety of non-tumorous pathologic conditions of the sternum and adjacent joints, with emphasis on CT, to help radiologists, particularly thoracic radiologists, to make an accurate diagnosis in their daily practice. Non-tumorous abnormalities include trauma (fractures and dislocations), infection (osteomyelitis, septic arthritis), degenerative (osteoarthritis) and inflammatory conditions (rheumatoid arthritis, seronegative arthritides), and metabolic disorders (Paget's disease and renal osteodystrophy) as well as treatment related changes such as poststernotomy and its complications (dehiscence, nonunion) and postradiation changes of the sternum.
Asunto(s)
Artritis , Fracturas Óseas , Dolor en el Pecho , Humanos , Esternón/anomalías , Esternón/diagnóstico por imagen , Esternón/lesiones , Tomografía Computarizada por Rayos X/efectos adversosRESUMEN
Sternal cleft is a rare malformation with significant morbidity and mortality. It has been associated with other midline fusion defects, most significantly Cantrell's pentalogy, involving the sternum, pericardium, heart, diaphragm, and abdominal wall. This study reported a successfully managed case of a newborn with a total sternal cleft and Cantrell's pentalogy. A review of literature and pertinent management principles was also conducted.
Asunto(s)
Cardiopatías Congénitas , Pentalogía de Cantrell , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Anomalías Musculoesqueléticas , Pentalogía de Cantrell/diagnóstico , Pentalogía de Cantrell/cirugía , Pericardio/anomalías , Pericardio/cirugía , Esternón/anomalías , Esternón/cirugíaRESUMEN
A neonate with thoracic ectopia cordis presented following an uncomplicated delivery. A mandibular distractor was placed to bridge the sternal cleft and retracted (reverse distractor activation) over 24 days to facilitate sternal closure. Follow-up at five years postoperatively demonstrated a well-healed sternum. This novel approach to ectopia cordis repair facilitates slow, steady physiologic accommodation of the heart without hemodynamic instability or long-term complications.
Asunto(s)
Ectopía Cordis , Cardiopatías Congénitas , Anomalías Musculoesqueléticas , Ectopía Cordis/diagnóstico por imagen , Ectopía Cordis/cirugía , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Esternón/anomalías , Esternón/diagnóstico por imagen , Esternón/cirugíaRESUMEN
INTRODUCTION: Pectus excavatum (PE) is a funnel-shaped indentation of the sternum and is the most common deformity of the chest wall. It is associated with syndromic diseases but can occur as an isolated form. Familial occurrence is assumed in up to 40% of cases, but large-scale studies are lacking. Most of the data are obtained from case reports which postulate autosomal recessive, dominant with reduced penetrance, X-linked, and multifactorial patterns of inheritance. No monogenetic cause has been identified to date. This study was designed to provide basic information on the epidemiology, family history, and comorbidity for a large cohort of isolated PE and to show that there is an inheritance pattern for PE that indicates a genetic background. MATERIALS AND METHODS: A retrospective study was done using a paper-based questionnaire for all PE patients attending two specialized centers for chest wall deformities. Patients with isolated PE were included and asked to provide information on family history and comorbidities. RESULTS: Family history was available for 78 patients. A positive family history was found in 42 patients (54%) with a total of 53 affected family members. CONCLUSION: The described family histories indicate an underlying genetic cause for PE. Identification of the genetic factors may contribute to characterize patients who are at risk of inheriting isolated PE.
Asunto(s)
Tórax en Embudo , Pared Torácica , Estudios de Cohortes , Tórax en Embudo/etiología , Tórax en Embudo/genética , Humanos , Estudios Retrospectivos , Esternón/anomalías , Pared Torácica/anomalíasRESUMEN
OBJECTIVES: To define the learning process of minimally invasive repair of pectus excavatum by the Nuss procedure through assessment of consecutive procedural metrics. METHODS: A single-center retrospective observational cohort study was conducted of all consecutive Nuss procedures performed by individual surgeons without previous experience between June 2006 and December 2018. Surgeons were proctored during their initial 10 procedures. The learning process after the proctoring period was evaluated using nonrisk-adjusted cumulative sum (ie, observed minus expected) failure charts of complications. An acceptable and unacceptable complication rate of 10% and 20% were used. Logarithmic trend lines were used to assess over-time performance regarding operation time. RESULTS: Two-hundred twenty-two consecutive Nuss procedures by 3 general thoracic surgeons were evaluated. Cumulative sum charts showed an average performance from the first procedure after being proctored onward for all surgeons, whereas surgeon B demonstrated a statistically significant complication rate equal to or less than 10% after 59 cases. Post-hoc sensitivity analyses using a stricter acceptable and unacceptable complication rate of 6% and 12% also showed an average performance for all surgeons. Although, the median time between consecutive procedures ranged from 7 to 35 days, no frequency-outcome relationship was observed. In addition, surgeons required the same average operation time throughout their entire experience. CONCLUSIONS: After a 10-procedure proctoring period, repair of pectus excavatum by the Nuss procedure is a safe procedure to adopt and perform without a typical (complication based) learning curve while performing at least 1 procedure per 35 days.