Pediatric chest ultrasound: a practical approach.

Chest ultrasonography is an important imaging adjunct for diagnosing and managing disease in children. Compared with CT and MRI, ultrasound is cheaper, portable and provides vascular or flow-related information that cannot otherwise be obtained noninvasively. The spatial and temporal resolution of ultrasound is excellent, particularly for superficial structures. In cases where a suspicious abnormality is found, tissue sampling can be performed percutaneously with US guidance. Ultrasound also excels at demonstrating and characterizing pleural fluid collections. As concerns about radiation exposure increase among laypersons and doctors alike, there is a compelling argument for making ultrasonography the initial imaging study of choice for many thoracic abnormalities in a child. In this review the authors discuss and illustrate the US findings of some of the more common chest complaints in children.

Diagnostic accuracy of post-mortem MRI for thoracic abnormalities in fetuses and children.

OBJECTIVES: To compare the diagnostic accuracy of post-mortem magnetic resonance imaging (PMMR) specifically for non-cardiac thoracic pathology in fetuses and children, compared with conventional autopsy. METHODS: Institutional ethics approval and parental consent was obtained. A total of 400 unselected fetuses and children underwent PMMR before conventional autopsy, reported blinded to the other dataset. RESULTS: Of 400 non-cardiac thoracic abnormalities, 113 (28 %) were found at autopsy. Overall sensitivity and specificity (95 % confidence interval) of PMMR for any thoracic pathology was poor at 39.6 % (31.0, 48.9) and 85.5 % (80.7, 89.2) respectively, with positive predictive value (PPV) 53.7 % (42.9, 64.0) and negative predictive value (NPV) 77.0 % (71.8, 81.4). Overall agreement was 71.8 % (67.1, 76.2). PMMR was most sensitive at detecting anatomical abnormalities, including pleural effusions and lung or thoracic hypoplasia, but particularly poor at detecting infection. CONCLUSIONS: PMMR currently has relatively poor diagnostic detection rates for the commonest intra-thoracic pathologies identified at autopsy in fetuses and children, including respiratory tract infection and diffuse alveolar haemorrhage. The reasonable NPV suggests that normal thoracic appearances at PMMR exclude the majority of important thoracic lesions at autopsy, and so could be useful in the context of minimally invasive autopsy for detecting non-cardiac thoracic abnormalities. KEY POINTS: • PMMR has relatively poor diagnostic detection rates for common intrathoracic pathology • The moderate NPV suggests that normal PMMR appearances exclude most important abnormalities • Lung sampling at autopsy remains the "gold standard" for pulmonary pathology.

Congenital kypho-scoliosis: a case of thoracic insufficiency syndrome and the limitations of treatment.

INTRODUCTION: Congenital spinal vertebral anomalies may present with deformity resulting in congenital scoliosis and kyphosis. This leads to abnormal spinal growth. The latter when combined with associated rib fusions may impair normal thoracic cage development and resultant pulmonary hypoplasia. Most congenital scoliosis can be detected in utero by ultrasound scan or recognized in the neonatal period, but a few spinal defects can remain undetected. MATERIALS AND METHODS: In this Grand Round, we present the case of a 7-year-old girl with a severe scoliosis and thoracic insufficiency syndrome (TIS). 3D CT reconstruction imaging demonstrated a mixed picture of fusion and segmentation abnormalities. A marked kyphoscoliosis was demonstrated at the thoraco-lumbar junction. Via a left thoracotomy, anterior excision of intervertebral discs was performed together with, interbody fusion, and in situ stabilisation of the kyphosis with double allograft (femur) strut grafts. CONCLUSIONS: This article highlights the features of congenital kypho-scoliosis and TIS. The difficulties of treating kyphosis when combined with TIS are discussed together with the limitations of current surgical techniques.

Isolated bifid rib: clinical and radiological findings in children.

BACKGROUND: Bifid rib is usually asymptomatic but sometimes occurs associated with other pathological conditions. The aim of this study was to investigate clinical and radiological characteristics in children with bifid rib. METHODS: Nine children with the diagnosis of bifid rib between 2005 and 2010 were reviewed. Chest radiography and computed tomography (CT) were performed in all patients, and 3-D images were additionally reconstructed in six patients. RESULTS: Five girls and four boys with a mean age at presentation of 4.2 years had various types of unilateral bifid rib. Seven patients complained of a chest wall mass, whereas two patients whose costal abnormalities were incidentally detected on chest radiography were asymptomatic. Bifid ribs were confirmed on plain radiographs in six patients, while the other three patients were finally diagnosed on reconstruction 3D-CT. One patient with a flared rib on a radiograph showed bifurcation of the costal cartilage on 3D-CT. The other two patients with upper rib abnormalities on radiography had downward extension of the cervical or first rib articulating with the upper branch of the bifid first or second rib, respectively. CONCLUSION: Reconstruction 3D-CT can demonstrate complicated thoracic abnormalities in patients with atypical appearance of the rib on plain radiographs. An isolated bifid rib may require no further intervention.

Radiological evaluation of dysmorphic thorax of paternal uniparental disomy 14.

BACKGROUND: The "coat-hanger" sign of the ribs with a bell-shaped thorax has been known as a radiological hallmark of the paternal uniparental disomy 14 (upd(14)pat). OBJECTIVE: To quantitatively determine the differences in thoracic deformity between upd(14)pat and other bone diseases with thoracic hypoplasia and to establish the age-dependent evolution. MATERIALS AND METHODS: The subjects comprised 11 children with upd(14)pat. The angle between the 6th posterior rib and the horizontal axis was measured (coat hanger angle; CHA). The ratio of the mid- to widest thorax diameter (M/W ratio) was calculated for the bell-shaped thorax. RESULTS: CHA ranged from +28.5 to 45° (mean; 35.1° ± 5.2) in upd(14)pat, and from -19.8 to 21° (-3.3 ± 13°) in bone dysplasias (p < 0.01). The M/W ratio ranged from 58% to 93% (75.4 ± 10) in upd(14)pat, and from 80% to 92% (86.8 ± 3.3) in bone dysplasias (p < 0.05). Serial radiographs revealed that CHA remained constant during early childhood, while the M/W ratio gradually increased with age. CONCLUSION: The "coat-hanger" sign of upd(14)pat provides a distinctive radiological gestalt that makes it possible to differentiate the disorder from other skeletal dysplasias. By contrast, the bell-shaped thorax is significant only in the neonatal period.

Fetal body MRI and its application to fetal and neonatal treatment: an illustrative review.

This Review depicts the evolving role of MRI in the diagnosis and prognostication of anomalies of the fetal body, here including head and neck, thorax, abdomen and spine. A review of the current literature on the latest developments in antenatal imaging for diagnosis and prognostication of congenital anomalies is coupled with illustrative cases in true radiological planes with viewable three-dimensional video models that show the potential of post-acquisition reconstruction protocols. We discuss the benefits and limitations of fetal MRI, from anomaly detection, to classification and prognostication, and defines the role of imaging in the decision to proceed to fetal intervention, across the breadth of included conditions. We also consider the current capabilities of ultrasound and explore how MRI and ultrasound can complement each other in the future of fetal imaging.

Ex utero intrapartum treatment (EXIT), a resuscitation option for intra-thoracic foetal pathologies.

The ex utero intrapartum treatment (EXIT) procedure is designed to guarantee sufficient oxygenation for a foetus at risk of airway obstruction. This is achieved by improving lung ventilation, usually by establishing an airway during caesarean delivery whilst preserving the foetal-placental circulation temporarily. Indications for the EXIT procedure have extended from its original use in reversing iatrogenic tracheal obstruction in congenital diaphragmatic hernia to naturally occurring upper airway obstructions. We report our experience with a new and rarely mentioned indication for the EXIT procedure, intra-thoracic volume expansions. The elaboration of lowest risk scenarios through balancing risks with alternative options, foetal or neonatal intervention and coordination between professionals from various disciplines are the most important conditions for a successful EXIT procedure. The EXIT procedure requires a caesarean section that specifically differs from the traditional caesarean section during which uterine tone is maintained to minimize maternal bleeding. To guarantee foetal oxygenation during the EXIT procedure, profound uterine relaxation is desired. To gain time with optimal placental oxygenation in order to safely perform an airway intervention in a baby at risk of hypoxia may require deep inhalation anaesthesia and/or tocolytic agents. We review the EXIT procedure and present a case series from the University Hospital of Geneva that contrasts with the common indication for the EXIT procedure usually based on upper airway obstruction by its exclusive indication for intra-thoracic malformations/diseases.

Thoracic Insufficiency Syndrome.

Thoracic insufficiency syndrome (TIS) is a broad grouping of disorders that have a substantial impact on the chest wall, spine, and in many situations, both. While the conditions are varied, they share a potentially substantial impact on respiratory capacity and development over time and a presentation and need for intervention that is often in early childhood. Addressing these conditions has required a new paradigm that involves both deformity correction and a preservation of growth capacity. While there are now a number of options to treat severe spinal deformity early in life, when the deformity causes or is caused by a chest wall deformity, the Vertical Expandable Prosthetic Titanium Rib(VEPTR) is able to support surgical correction of both. The skeletal correction is often quite dramatic, but the functional measurements of quality of life and pulmonary function often do not show as dramatic and improvement, but consistently show a stabilization indicating control of the progressive thoracospinal disorder that produced TIS.

Imaging of congenital chest wall deformities.

To identify the anatomy and pathology of chest wall malformations presenting for consideration for corrective surgery or as a possible chest wall "mass", and to review the common corrective surgical procedures. Congenital chest wall deformities are caused by anomalies of chest wall growth, leading to sternal depression or protrusion, or are related to failure of normal spine or rib development. Cross-sectional imaging allows appreciation not only of the involved structures but also assessment of the degree of displacement or deformity of adjacent but otherwise normal structures and differentiation between anatomical deformity and neoplasia. In some cases, CT is also useful for surgical planning. The use of three-dimensional reconstructions, utilizing a low-dose technique, provides important information for the surgeon to discuss the nature of anatomical abnormalities and planned corrections with the patient and often with their parents. In this pictorial essay, we discuss the radiological features of the commonest congenital chest wall deformities and illustrate pre- and post-surgical appearances for those undergoing surgical correction.

An approach to the asymmetric neonatal chest radiograph.

Conditions causing respiratory distress in the neonate, which may require surgery or an interventional procedure, typically have asymmetric radiographic findings. The findings are conveniently categorized from the plain radiographs as those producing a large lucent-appearing hemithorax, those producing a large cystic-appearing hemithorax, and those producing a large opaque-appearing hemithorax. Additional imaging with ultrasound, CT, MR imaging, or fluoroscopy can be planned based on the initial radiographic appearance.

Prenatal diagnosis and management of fetal thoracic lesions.

There have been significant strides made during the last decade in understanding the natural history and pathophysiology of fetal thoracic lesions. Largely as a result of advances in prenatal ultrasound, we are not only able to diagnose these lesions and advise parents about prognosis, but also offer the possibility of fetal intervention for the most severely affected fetuses. However, large gaps remain in the current state of knowledge of fetal thoracic lesions. We are unable to accurately predict pulmonary hypoplasia, the most devastating consequence of fetal thoracic lesions. In lesions, such as CDH, the selection criteria for fetal intervention remain ill defined. Proof of the efficacy and superiority of fetal surgery over conventional postnatal therapies for diaphragmatic hernia await the results of prospective trials. Fetal surgery in lesions such as CCAM and BPS is currently reserved for only those fetuses with hydrops and a uniformly fatal outcome. Whether fetal surgery in these cases would be beneficial in the absence of hydrops is difficult to say especially given the possibility of spontaneous regression. During the next decade we anticipate continued growth in our understanding of these lesions, refinement in selection criteria for intervention, and advances in techniques for salvaging these severely compromised fetuses. The diagnosis and treatment of fetal thoracic lesions remains a formidable challenge, but one which can be met with cautious optimism, due to the availability of fetal interventions not previously available.

Multilevel thoracic pedicle aplasia causing congenital thoracic kyphosis: case report.

OBJECTIVE AND IMPORTANCE: Congenital thoracic kyphosis is a rare cause of treatable myelopathy. Multilevel thoracic pedicle aplasia as a cause of this deformity has not been previously reported in the literature. We report a case and describe the surgical management and outcome. CLINICAL PRESENTATION: A 14-year-old boy presented to us with a 4-month history of back pain and slowly progressive spastic paraparesis. Radiographic studies revealed thoracic kyphosis and bilateral aplasia of the pedicles of T4-T8. INTERVENTION: The patient underwent surgical treatment via a posterior approach for decompression of T4-T8, followed by arthrodesis from T2 to T12, using a hook claw construct with multiple points of fixation and autologous bone grafting. CONCLUSION: Congenital vertebral anomalies may be clinically occult, and delayed presentation may occur in adolescence or adulthood. Aplasia of multiple thoracic pedicles can produce kyphotic deformities with neurological compromise. A posterior approach with multiple points of segmental instrumentation can be effective in treating kyphotic deformities that are flexible and of moderate severity (<75 degrees).

Congenital chest wall mesenchymal hamartoma.

The case of an infant with mesenchymal hamartoma of the chest wall is presented, and the role of conservative diagnostic and therapeutic intervention is emphasized. A large mass that involved the right hemithorax and chest wall was detected in utero on routine ultrasonographic studies and diagnosed as mesenchymal hamartoma by percutaneous fine needle biopsy at 4 days after birth. The mass did not enlarge after birth, but surgical debulking was necessary at 1 month because of progressive respiratory compromise. Tumor regrowth was noted over the ensuing 8 months, and a second debulking at 9 months has been followed by a 6-year interval without evidence of recurrence.

Multifocal mesenchymal hamartoma of the chest wall in infancy.

The authors report on an infant who had multifocal mesenchymal hamartoma of the right posterior chest wall. The tumors were found incidentally, on a chest radiograph, during routine evaluation for upper respiratory tract infection. Resection of both lesions with chest wall reconstruction was performed, with a good result. Only 46 cases of this unusual tumor have been reported previously, and only two of them were multifocal.

A case of ruptured descending thoracic aortic aneurysm into the right pleural cavity: importance of preoperative drainage of the right pleural cavity.

We present an unusual case of a ruptured descending thoracic aortic aneurysm into the right pleural cavity of a patient with pectus carinatum. The presence of pectus carinatum played an important role in the development of the aneurysm at the atypical site and the rupture into the right pleural cavity. A small amount of right pleural bleeding on admission can increase and develop to massive hemothorax until emergency operation. Massive bleeding in the right pleural cavity where the dependent lung is located causes atelectasis and increased shunt fraction under one lung ventilation. Therefore, continuous drainage of the right pleural cavity is essential to prevent serious hypoxia during graft replacement in a case of ruptured descending thoracic aneurysm into the right hemithorax.

[Tumors and cystic formations in the chest cavity of children]. / Opukholi i kistoznye obrazovaniia v grudnoi polosti u detei.

During 13 years 91 children with sizable formations of the lungs and mediastinum aged from 3 months up to 15 years were treated in the clinic. The rationally selected volume of clinical examinations facilitated differential diagnosis in the preoperative period and excluded diseases simulating sizable processes (intrathoracic dystopia of the kidney, malformations of the vessels etc.). An incomplete examination of such patients might result in diagnostic errors followed by unjustified operative interventions.

Chest wall repair with engineered fetal bone grafts: an efficacy analysis in an autologous leporine model.

PURPOSE: We sought to compare the efficacy of engineered fetal bone grafts with acellular constructs in an autologous model of chest wall repair. METHODS: Rabbits (n = 10) with a full-thickness sternal defect were equally divided in 2 groups based on how the defect was repaired, namely, either with an autologous bone construct engineered with amniotic mesenchymal stem cells on a nanofibrous scaffold or a size-matched identical scaffold with no cells. Animals were killed at comparable time-points 18 to 20 weeks postimplantation for multiple analyses. RESULTS: Gross evidence of nonunion confirmed by micro-computed tomography scanning was present in 3 (60%) of 5 of the acellular implants but in no engineered grafts. Histology confirmed the presence of bone in both types of repair, albeit seemingly less robust in the acellular grafts. Mineral density in vivo was significantly higher in engineered grafts than in acellular ones, with more variability among the latter. There was no difference in alkaline phosphatase activity between the groups. CONCLUSIONS: Chest wall repair with an autologous osseous graft engineered with amniotic mesenchymal stem cells leads to improved and more consistent outcomes in the midterm when compared with an equivalent acellular prosthetic repair in a leporine model. Amniotic fluid-derived engineered bone may become a practical alternative for perinatal chest wall reconstruction.