Novel Clinical Algorithm for Prenatal Monitoring of Congenital Lung Malformations.

INTRODUCTION: Congenital lung malformations (CLMs) are readily identified early in pregnancy with a variable natural history. Monitoring for lesion size and mediastinal shift (MS) is recommended following diagnosis. The purpose of this study is to propose a risk-stratified clinical algorithm for prenatal monitoring of CLM. METHODS: After ethical approval, all fetuses with CLMs evaluated at our fetal center from January 2015 to June 2022 were retrospectively reviewed. Patient demographics, imaging characteristics, and fetal interventions were collected. Lesions were stratified by congenital lung malformation volume ratio (CVR) and the presence of MS. Descriptive statistics and receiver operating characteristic curves were employed in the analysis. RESULTS: We analyzed 111 patients with a mean of 23.4 wk gestational age, a median CVR of 0.5 (interquartile range, 0.3-1.2), and MS in 76 of 111(68%) patients on initial evaluation. Among low-risk patients (CVR ≤1.1), 96% remained low-risk on final evaluation. No patients transitioned from low to high risk during the growth period. Patients with CVR >1.1 often had persistent MS (P < 0.001). Hydrops (5/111, 5%) and fetal intervention (4/111, 4%) only occurred in patients with CVR >1.1 (P < 0.001, P = 0.002) and MS (P = 0.144, P = 0.214). On receiver operating characteristic curve analysis, initial CVR >1.1 had 100% sensitivity and negative predictive value for hydrops and fetal intervention. CONCLUSIONS: CLMs with initial CVR ≤1.1 are low risk for hydrops and fetal intervention. We propose a risk-stratified algorithm for the monitoring of CLM during the growth period based on CVR. While our experience suggests that patients with CLM and MS are at higher risk, the current subjective assessment of MS is not adequately predictive. Incorporating an MS grading system may further refine risk stratification in the management of CLM.

Cardiac Computed Tomographic Angiography Evaluation of Right Ventricle-Pulmonary Artery Conduits with Surgical Correlation.

Right ventricle-pulmonary artery (RV-PA) conduits are used in the treatment of certain congenital heart disease (CHD). RV-PA conduit complications might develop over time and require intervention. To evaluate how well cardiac computed tomographic angiography (CCTA) performs compared to transthoracic echocardiography (TTE) in evaluating RV-PA conduit complications by using surgical findings as the reference standard. A retrospective chart review of all patients over a 5-year period who underwent CCTA for RV-PA conduit evaluation was performed. Patient demographics and clinical data were recorded. Preoperative CCTA and TTE findings were compared to the operative findings for concordance or discordance. Forty-one patients were included, 51% females. The complications were conduit stenosis (28.68%), infection (7.17%) and aneurysm/pseudoaneurysm (6.15%). TTE and CCTA were consistently able to visualize focal conduit stenosis (96%). The greatest discrepancy between TTE and CCTA was in evaluating for aneurysm/pseudoaneurysm, where TTE detected only 2/6 (33%) compared to CCTA which detected 6/6 (100%) of the cases. However, TTE was slightly better at detecting conduit infection (3/7, 43%) compared to CCTA (2/7, 29%). Note that 5 out of 7 patients with endocarditis had bovine jugular graft. CCTA and TTE provide similar diagnostic accuracy evaluating certain types of RV-PA conduit complications. However, certain complications were only visualized on CCTA or TTE making both modalities complementary to each other during diagnostic evaluation.

Role of gated cardiac computed tomographic angiography in the evaluation of postsurgical complications after stage I Norwood procedure and its implications on management: a comparative study with two-dimensional echocardiography.

BACKGROUND: The Norwood procedure is the first part of a three-stage surgical palliation for patients with functionally single ventricle anatomy. Complications after the stage I operation are not uncommon. Transthoracic echocardiography (TTE) is traditionally the mainstay for evaluation. OBJECTIVE: The purpose of our study is to compare gated cardiac computed tomographic angiography (CCTA) with TTE when evaluating for postoperative complications after stage I Norwood procedure and to describe management implications. MATERIALS AND METHODS: A retrospective chart review of all patients over a 4-year period who underwent nonelective urgent CCTA for suspected complications related to stage I Norwood procedure was performed. Elective CCTA studies before stage II palliation were excluded. Patient demographics, CCTA and TTE findings, as well as interventions performed, were recorded. RESULTS: Thirty-four patients were included. The mean age at CCTA was 63 days (range: 4-210 days). All patients had a recent TTE with a mean time interval between TTE and CCTA of 2 days. CCTA detected 56 abnormalities in 30 patients, with 23 directly related to postsurgical complications, including shunt-related complications (10/23, 43%), Damus-Kaye-Stansel anastomotic narrowing (2/23, 9%) and neo-aortic arch/branch vessel abnormalities (11/23, 48%). These complications were managed as follows: surgery (9, 39%), catheter-based intervention (7, 30%), medical (4, 17%) and no change in management (3, 13%). TTE did not detect 8/23 (35%) findings found on CCTA, of which 75% were either managed with surgery (4/8, 50%) or catheter-based intervention (2/8, 25%). CONCLUSION: CCTA plays an important role in detecting surgical complications after stage I Norwood procedure and demonstrates additional findings that have direct management implications.

Value of emergent pediatric cardiac computed tomographic angiography service: initial experience at a large children's hospital.

BACKGROUND: Demand for pediatric cardiac computed tomography (CT) angiography is increasing due to recent advances that reduce the need for sedation and radiation exposure while enhancing diagnostic accuracy. This has resulted in the increasing use of cardiac CT angiography emergently during weekends and after hours. The unexpected demand for these services can be challenging, as most hospitals are not staffed to provide 24/7 pediatric cardiovascular imaging. OBJECTIVE: To describe a large single-center experience of providing emergent cardiac CT angiography services in children. MATERIALS AND METHODS: We identified all patients who underwent after--hours weekday and weekend emergent cardiac CT angiography between January 2017 and August 2018. Cardiac CT angiography in the settings of congenital heart disease and coronary imaging were included. Data collected included day and time of cardiac CT angiography, patient age, referral unit, indication, surgical history, need for sedation, need for surgery, intervention and/or change in medical management based on the cardiac CT angiography. RESULTS: Forty-seven studies were identified, 26 (55%) of which were performed on a weekend or holiday and 21 (45%) after 5 p.m. on a weekday. Based on cardiac CT angiography findings, 20 (43%) patients underwent either surgery or an interventional procedure, and 9 (19%) had a change in medical management. The time between cardiac CT angiography and the related surgery/intervention ranged from 0 to 29 days with a median of 3.5 days. CONCLUSION: Emergent pediatric cardiac CT angiography is a valuable service. Larger multi-institutional studies with standardized referral and utilization patterns are needed to determine if outcomes are affected by this service, which in turn will influence hospital staffing patterns for emergent imaging.

A novel approach using volumetric dynamic airway computed tomography to determine positive end-expiratory pressure (PEEP) settings to maintain airway patency in ventilated infants with bronchopulmonary dysplasia.

BACKGROUND: Positive end-expiratory pressure (PEEP) is a key mechanical ventilator setting in infants with bronchopulmonary dysplasia (BPD). Excessive PEEP can result in insufficient carbon dioxide elimination and lung damage, while insufficient PEEP can result in impaired gas exchange secondary to airway and alveolar collapse. Determining PEEP settings based on clinical parameters alone is challenging and variable. OBJECTIVE: The purpose of this study was to describe our experience using dynamic airway CT to determine the lowest PEEP setting sufficient to maintain expiratory central airway patency of at least 50% of the inspiratory cross-sectional area in children with BPD requiring long-term ventilator support. MATERIALS AND METHODS: We retrospectively identified all infants with BPD who underwent volumetric CT with a dynamic airway protocol for PEEP optimization from December 2014 through April 2019. Sixteen infants with BPD underwent 17 CT exams. Each CT exam consisted of acquisitions spanning the trachea and mainstem bronchi. We measured cross-sectional area of the trachea and mainstem bronchi and qualitatively assessed the amount of atelectasis. We documented changes in management as a result of the CT exam. RESULTS: The average effective dose was 0.1-0.8 mSv/scan. Of 17 CT exams, PEEP was increased in 9, decreased in 3 and unchanged after 5 exams. CONCLUSION: Dynamic airway CT shows promise to assist the clinician in determining PEEP settings to maintain airway patency in infants with BPD requiring long-term ventilator support. Further evaluation of the impact of this maneuver on gas exchange, cardiac output and other physiological measures is needed.

Body MR angiography in children: how we do it.

Vascular pathology is ubiquitous in children. Common indications for angiographic imaging in the body include congenital anomalies, portal hypertension, assessing resectability of neoplasms, renovascular hypertension, vascular malformations, vasculitis, systemic vein thrombosis, and trauma. MR angiography, with or without the use of intravenous contrast agents, is therefore a mainstay in the repertoire of MR imaging in children. Pediatric contrast-enhanced MR angiography has benefited from several innovations in recent years, including improved hardware options like high-field-strength scanners and integrated high-density coil arrays, new sequences that combine parallel imaging, innovative k-space sampling and Dixon fat suppression with time-resolved imaging, new contrast agents with longer blood-pool residence time, and advanced post-processing solutions like image fusion. This article focuses on the principles of contrast-enhanced MR angiography of the body as it pertains to the physiologies and pathologies encountered in children. It also discusses tools to adapt the MR angiographic technique to the clinical indication, as well as pitfalls of post-processing and interpretation in commonly encountered vascular imaging scenarios in the pediatric body.

Congenital Chest Lesions and Interventions.

Anomalies of the fetal chest require advanced imaging with ultrasound and MR imaging as well as expertise on the part of the interpreting pediatric radiologist. Congenital diaphragmatic hernia and congenital lung malformation are the most frequently seen, and in both conditions, the radiologist should provide both detailed anatomic description and measurement data for prognostication. This article provides a detailed approach to imaging the anatomy, in-depth explanation of available measurements and prognostic value, and keys to identifying candidates for fetal intervention. Less common congenital lung tumors and mediastinal and chest wall masses are also reviewed.

Detection of anomalous aortic origin of a coronary artery (AAOCA) by echocardiogram: When does computed tomographic angiography add value?

BACKGROUND AND OBJECTIVE: There is limited literature comparing TTE and CCTA in children with suspected AAOCA. To determine the distribution of various coronary anomalies comparing TTE and CCTA data, and define the added value advanced imaging brings in clinical decision-making. MATERIALS AND METHODS: Retrospective review of data was obtained in patients aged 0-18 years who underwent TTE and CCTA for suspected AAOCA. Patient demographics, CCTA and TTE findings, and interventions performed were recorded. RESULTS: 100 consecutive patients were included (60% male), mean age 11 years (7 days-18 years old). In 93 patients, CCTA detected 94 anomalous coronaries. Definitive coronary abnormality was reported on TTE in 77 patients; 76 of which were confirmed by CCTA, 1 patient was found to have a normal variant. Suspected anomalous origin was reported in 16 patients on TTE, 13 of which were abnormal on CCTA. The coronary origin was not seen on TTE in 6 patients; of these, 3 had AAOCA on CCTA and 3 had hypoplastic RCA with left dominant system. Only 1 patient who had a normal TTE was found to have AAOCA on CCTA. CCTA was better than TTE in defining ostial characteristics and the course of the anomalous coronary artery, and detecting myocardial bridge. CONCLUSIONS: CCTA adds value in diagnosing AAOCA when the coronary origins are not well assessed or suspected anomalous origin is suggested on TTE. In addition, when a confident definitive diagnosis of AAOCA is reported on TTE, CCTA demonstrates better performance in determining additional features of AAOCA.

Fetal malrotation with midgut volvulus: Prenatal diagnosis and planning.

Introduction: Malrotation of the intestinal tract is a congenital malformation commonly found either incidentally or after affected individuals develop signs and symptoms of intestinal obstruction. Malrotation is prone to midgut volvulus that can cause intestinal obstruction and lead to ischemia and necrosis requiring emergent surgical intervention. Rare instances of in utero midgut volvulus have been reported in the literature and carry a high mortality given the difficulty in establishing a diagnosis prior to development of signs of intestinal ischemia and necrosis. Advancements in imaging have made it possible to diagnose in utero malrotation earlier, raising the question of optimal timing of delivery, especially in cases of prenatally diagnosed midgut volvulus. In these cases, the risks of premature birth must be weighed against the risks of fetal intestinal ischemia and potential fetal demise. Case presentation: This case report details an interesting presentation of intestinal malrotation with suspected midgut volvulus found on prenatal imaging at 33 weeks and 4 days' gestation. This prompted delivery of the infant at 34 weeks and 2 days' gestation with urgent operative management, within 3 hours of life, after diagnosis was confirmed postnatally. Intraoperatively, the infant was confirmed to have midgut volvulus without bowel ischemia, the intestines were reduced, and a Ladd procedure was performed without incident. The infant recovered postoperatively without complication, tolerated advancement to full volume feeds and was discharged on day of life 18. Conclusion: Successful management of fetal malrotation with midgut volvulus may be accomplished by early access to a multi-disciplinary team of professionals, prompt postnatal confirmation of diagnosis, and urgent correction to minimize the risk of complications.

Case Report: The Medical and Surgical Management of an Infant With Extreme Prematurity and Fetus-In-Fetu.

Fetus-in-fetu (FIF) is a rare congenital anomaly where a parasitic twin is within the body of a host twin. FIF is reported to occur in 1:500,000 live births. Herein, we report the first case of the medical and surgical treatment of a FIF patient who was born with extreme prematurity at 25-weeks gestation. With the multi-disciplinary coordination of neonatology, surgery, and interventional radiology, the patient was able to achieve a window of medical stability 4 weeks after birth. A decision was made at that time to proceed with an intra-abdominal and perineal resection of the FIF. The FIF was successfully resected and the patient was able to recover from the operation, with eventual discharge from the NICU. In conclusion, extreme prematurity and FIF may be amenable to surgical resection and a multi-disciplinary approach is crucial to achieve the desired outcome.

Pulmonary Embolism After Vaginal Delivery in a Fontan Patient.

The Fontan procedure was created to address the mixing of pulmonary and systemic venous return in patients with a single functional ventricle. The patient in this case with a Fontan repair experienced multiple pulmonary emboli 10 days post-partum. We outline management and recommendations when treating these patients. (Level of Difficulty: Beginner.).

Prenatal diagnosis and assessment of congenital spinal anomalies: Review for prenatal counseling.

The last two decades have seen continuous advances in prenatal ultrasonography and in utero magnetic resonance imaging. These technologies have increasingly enabled the identification of various spinal pathologies during early stages of gestation. The purpose of this paper is to review the range of fetal spine anomalies and their management, with the goal of improving the clinician's ability to counsel expectant parents prenatally.

Prenatal Imaging of Craniosynostosis Syndromes.

This article reviews the prenatal diagnosis of those syndromes in which craniosynostosis is a key feature. Although not an exhaustive list, the authors highlight conditions that may be encountered with some regularity, especially in a higher volume fetal imaging center. Rare conditions are also discussed. Normal sutural anatomy and development are first reviewed, followed by a discussion of specific syndromes, the salient imaging findings, and pathologic as well as postnatal correlations when possible.