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.

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.

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.

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.

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.

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.

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.

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.

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.

Congenital thoracic vascular anomalies.

Congenital vascular anomalies of the thorax represent an important group of entities that can occur either in isolation or in association with different forms of congenital heart disease. It is extremely important that radiologists have a clear understanding of these entities, their imaging characteristics, and their clinical relevance. The imaging armamentarium available to diagnose these diverse conditions is ample, and has evolved from such traditional methods as chest radiography, barium esophagography, and angiography to new modalities that include echocardiography, multidetector row CT (MDCT), and MR imaging. These imaging modalities have added safety, speed, and superb resolution in diagnosis and, as in the case of MDCT, provide additional information about the airway and lung parenchyma, resulting in a more comprehensive examination with greater anatomic coverage. This article reviews the most important congenital thoracic vascular anomalies, their embryologic foundation, clinical presentation, and imaging characteristics, especially those of MDCT.

Prenatal presentation and postnatal management of congenital thoracic malformations.

The antenatal finding of a congenital thoracic malformation (CTM) leads to anxiety in the parents and uncertainty as to the optimal management. The antenatal spectrum of CTM includes congenital cystic adenomatoid malformation, sequestration, congenital lobar emphysema, enteric and bronchogenic cysts, and bronchial atresia. Most lesions require no antenatal intervention, and shrink substantially in the third trimester, but if fetal hydrops develops, then antenatal intervention is required, occasionally medical but more usually surgical, because mortality is high. If the baby is symptomatic in the newborn period, then some form of surgical intervention is clearly required. The asymptomatic baby presents a therapeutic dilemma. Advocates of early surgery point to the complications of CTM, which include infection, pneumothorax, bleeding and malignant transformation. Those who are proponents of conservative management retort that some CTM disappear postnatally, and that the complication rate is unknown; many children appear never to need surgery. Furthermore, there is clearcut evidence that excision of a CTM does not totally eliminate the risk of a subsequent malignancy. It is clear that, both antenatally and postnatally, counselling of the family on a case by case basis is needed. The limitations of present evidence should be stressed. Different families will make different decisions about postnatal surgery in an asymptomatic baby. If surgery is performed, morbidity is low, particularly with a video-assisted thoracoscopic (VATS) procedure.

Airway compression by the ascending aorta due to a thin thoracic cage.

An 89-year-old woman with dyspnea and disturbed consciousness due to hypoventilation was admitted to our hospital. Chest radiography showed no abnormal shadow, but she was intubated for deteriorated hypoventilation. Bronchoscopy demonstrated obstruction of the left main bronchus at the carina. Computed tomography (CT) showed neither a mass lesion in the mediastinum nor an aortic aneurysm, but compression of the airway by the ascending aorta was observed. It was thought that the patient's thin thoracic cage was unable to support the weight of the ascending aorta, which consequently compressed the left main bronchus. After inserting stents into both main bronchi, the patient's consciousness improved, and respirator support was withdrawn. In aged, bedridden, thin patients with hypopnea or recurrent airway infection, CT and bronchoscopy should be performed to investigate airway patency.

Hamartoma torácico congénito: a propóstio de un caso / Congenital thoracic hamartoma: A case report

A pesar de su poca frecuencia las tumoraciones de la cavidad torácica fetal constituyen casos realmente curiosos descritos en la literatura mundial. Dentro de estos los hamartomas, los linfangiomas, tumores fibrosos y teratomas mediastinales han sido reportados según hallazgos clínicos o de necropsia, pues no obstante su histología inequívocamente benigna puede acompañarse de otras malformaciones como el secuestro pulmonar, la hipoplasia pulmonar, pulmones quísticos, hidrops fetalis e hidrotórax. En este trabajo presentamos un hallazgo de la necropsia realizada a un feto masculino de 1360 g y 27 semanas de edad gestacional procedente de una interrupción de embarazo por diagnóstico prenatal de hernia diafragmática derecha, observándose durante la necropsia integridad del músculo diafragmático y la presencia de un tumor sólido y pediculado en la cavidad torácica derecha con estudio histológico concluyente de un hamartoma, acompañado de una hipoplasia moderada del pulmón derecho(AU)

In spite of its scarce frequency tumors of the fetal thoracic cavity constitute weird cases described in the world literature. Among them hamartomas, lymphangiomas, fibrosis tumors and mediastinal teratomas have been reported according to clinical or necropsy findings because no matter the undoubtedly benign histology, they may be together with some other anomalies, such as, the pulmonary sequestrum, pulmonary hypoplasia, cystic lungs, hydrops fetalis and hydrothorax. In this work it is presented the necropsy finding of a male fetus of 1360 grams and 27 weeks of gestation interrupted by the prenatal diagnosis of right diaphragmatic hernia, noticing during the necropsy, the integrity of the diaphragmatic muscle and the presence of a solid and pediculated tumor in the right thoracic cavity with a final histological research of harmatoma joined by moderated right lung hypoplasia(EU)

[Clinical characteristics of Bochdalek hernia in neonates and infants].

OBJECTIVE: To summarize the clinical characteristics, diagnosis and treatment of Bochdalek hernia in neonates and infants. METHODS: The data of 15 neonates and 10 infants with Bochdalek hernia,undergoing the normal diagnosis and surgical repair from August 1983 to June 2004, were retrospectively reviewed. Location was left in 22 cases and right in 3 cases. Twenty-four cases were treated by operation and 1 case died of respiratory failure before operation. RESULTS: Before April 1998, 7 of 8 (5 neonates, 3 infants) cases of Bochdalek hernia stayed healthy and respiratory symptom-free 1 year after operation; they were followed up 1 year and 3 months to 11 years. One premature neonate with Bochdalek hernia died of respiratory failure before operation, and his lung volume was found to be dysplasia. From April 1998 to June 2004, 15 (8 neonates,7 infants) of 17 (10 neonates, 7 infants)cases of Bochdalek hernia survived postoperatively, while 2 neonates died of respiratory failure. CONCLUSION: The earlier dyspnoea of neonates of Bochdalek hernia occur, the worse their healthy status appear. The standard and timely surgical repairs could improve the curative ratio. Whether the operation was suspended depended on the healthy states of babies.