Invited commentary for: Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle.

Thousands of neonates are born each year with complex congenital heart defects, such as total anomalous pulmonary venous connection combined with single ventricle physiology. This dual diagnosis with significant vessel and ventricular complexity requires alternative additional imaging to fully visualize the anatomical challenge and devise the appropriate treatment strategy for the patient.

Bleeding Complications in Neonates Receiving Extracorporeal Membrane Oxygenation and Controlled Hypothermia.

OBJECTIVE: Safety and efficacy data on controlled hypothermia (CH) for neonates with moderate to severe hypoxic ischemic encephalopathy has been extrapolated to a subgroup of these patients who also require extracorporeal membrane oxygenation for refractory persistent pulmonary hypertension of the newborn (PPHN). However, safety data on the concomitant use of CH and extracorporeal membrane oxygenation (ECMO) are lacking. METHODS: This is a single-center retrospective study of neonates ≥35 weeks' gestation with refractory PPHN who required ECMO between January 2010 and December 2020. Study groups were divided into those receiving CH/ECMO versus ECMO only. Baseline characteristics, short-term outcomes, and brain magnetic resonance imaging (MRI) data were compared. RESULTS: A total of 36 neonates who received ECMO for refractory PPHN were included. Of these, 44.4% (n = 16) received CH/ECMO and 55.6% (n = 20) received ECMO only. Bleeding complications were more common in CH/ECMO group 50% (n = 8) versus ECMO only 15% (n = 3, p = 0.023). T1 brain MRI severity scores were higher in CH/ECMO group versus ECMO only group, however, there were no statistical difference in T2 and diffusion-weighted image scores. Functional status and survival to discharge were comparable between groups. CONCLUSION: In our cohort, neonates who received CH/ECMO had higher bleeding complications than ECMO only group with comparable functional status and survival at discharge. KEY POINTS: · Safety data on the concomitant use of CH and ECMO are lacking in neonates.. · In our cohort, neonates who received CH/ECMO had higher bleeding complications than ECMO only group.. · Functional status and survival to discharge were no differences between the two groups..

Effect of radiologist pregnancy on the performance of pediatric fluoroscopic studies: a survey of Society for Pediatric Radiology members.

BACKGROUND: To our knowledge, the fluoroscopic practice patterns of pregnant diagnostic radiologists have not been documented. OBJECTIVE: To describe the fluoroscopic practice patterns during pregnancy among members of the Society for Pediatric Radiology (SPR) and potential impact on career and professional relationships. MATERIALS AND METHODS: After approval from the SPR, we sent all 1,847 SPR members an email link to an anonymous online survey that queried how pregnancy affects a practicing radiologist who performs fluoroscopy. RESULTS: Of the 398 responses (65% female, 35% male), most female respondents (78%) reported having been pregnant while practicing radiology and the majority (85%) performed fluoroscopy during pregnancy. Most performed fluoroscopy during all three trimesters (first 78%, second 90%, third 87%) and the majority (81%) used double-lead. Forty-six percent indicated that their fluoroscopic responsibilities during pregnancy were stressful. Of those who opted to perform fewer fluoroscopy studies, 20% indicated that it impacted negatively on their career or professional relationships. After witnessing a pregnant co-worker perform fewer fluoroscopic studies, 11% (4% male; 16% female; P<0.05) of respondents observed a subsequent negative impact on the co-worker's career or professional relationships. CONCLUSION: The majority of responding female SPR members had performed fluoroscopy during pregnancy and most performed fluoroscopy during their entire pregnancy with double-lead. Differences between self-reported adversity and observed adversity of performing less fluoroscopy during pregnancy speak to the lack of awareness of the issues, particularly among male colleagues. A clear policy with regard to performing fluoroscopy during pregnancy for all radiologists in every department is necessary.

Identification of clinical parameters to increase the diagnostic yield of the non-emergent upper gastrointestinal series in pediatric outpatients.

BACKGROUND: Outpatient, non-emergent upper gastrointestinal (GI) series are frequently requested in children with no surgical history who have nonspecific symptoms such as abdominal pain, failure to thrive and vomiting. The positive yield of an upper GI series in these patients, and, thus, its utility, has not been studied. OBJECTIVES: We evaluated the incidence of positive upper GI findings in children without a history of GI pathology or abdominal surgery in order to identify clinical indications associated with a greater diagnostic yield. MATERIALS AND METHODS: Findings of upper GI series performed between October 2015 and October 2017 in three institutions in children younger than 18 years of age were retrospectively reviewed. The upper GI series protocol for each institution was also reviewed. Children with a medical or surgical GI history, children with insufficient history in the chart and those with an incomplete upper GI series were excluded from the study. Exam indications, patient demographics and clinical history were obtained from the electronic medical records. RESULTS: Of 1,267 children who underwent outpatient upper GI series, 720 (median age: 2 years) had no GI history and were included in the study. The most common indications were non-bilious vomiting (62%), reflux symptoms (28%) and abdominal pain (20%). Upper GI series were normal in 605/720 cases (84%), including 25/26 children with reported bilious emesis. Of the 115 positive studies, 78 (68%) showed only gastroesophageal reflux (GER) (median age: 11 months). Of the remaining 37 studies, 19 demonstrated esophageal findings. One case of malrotation without midgut volvulus was identified in a patient who presented with dysphagia and reflux symptoms. Using a multinomial logistic regression model and adjusting for other variables, reflux symptoms and younger patient age were independent predictors of GER on upper GI series (relative risk ratios of 2.2 and 0.9, respectively). Dysphagia and/or foreign body sensation and older patient age were independent predictors of the presence of esophageal findings (relative risk ratios of 3.3 and 1.1, respectively). CONCLUSION: The yield of routine upper GI series in children with nonspecific symptoms, such as abdominal pain and vomiting, and no surgical history is low. Diagnostic yield was improved in older children and in those complaining of dysphagia and/or foreign body sensation. Routine upper GI series should be avoided in clinically well children with symptoms only of uncomplicated GER and no significant GI history. In children with a history of dysphagia and/or foreign body sensation, an esophagram/barium swallow can suffice.

Pediatric Temporal Bone Fractures: A 10-Year Experience.

OBJECTIVE: The aim of the study was to compare the traditional and newer temporal bone fracture classification systems and their reliability in predicting serious outcomes of hearing loss and facial nerve (FN) injury. METHODS: We queried the medical record database for hospital visits from 2002 to 2013 related to the search term temporal. A total of 1144 records were identified, and of these, 46 records with documented temporal bone fractures were reviewed for patient age, etiology and classification of the temporal bone fracture, FN examination, and hearing status. Of these records, radiology images were available for 38 patients and 40 fractures. RESULTS: Thirty-eight patients with accessible radiologic studies, aged 10 months to 16 years, were identified as having 40 temporal bone fractures for which the otolaryngology service was consulted. Twenty fractures (50.0%) were classified as longitudinal, 5 (12.5%) as transverse, and 15 (37.5%) as mixed. Using the otic capsule sparing (OCS)/violating nomenclature, 32 (80.0%) of fractures were classified as OCS, 2 (5.0%) otic capsule violating (OCV), and 6 (15.0%) could not be classified using this system. The otic capsule was involved in 1 (5%) of the longitudinal fractures, none of the transverse fractures, and 1 (6.7%) of the mixed fractures. Sensorineural hearing loss was found in only 2 fractures (5.0%) and conductive hearing loss (CHL) in 6 fractures (15.0%). Two fractures (5.0%) had ipsilateral facial palsy but no visualized fracture through the course of the FN canal. Neither the longitudinal/transverse/mixed nor OCS/OCV classifications were predictors of sensorineural hearing loss (SNHL), CHL, or FN involvement by Fisher exact statistical analysis (for SNHL: P = 0.37 vs 0.16; for CHL: P = 0.71 vs 0.33; for FN: P = 0.62 vs 0.94, respectively). CONCLUSIONS: In this large pediatric series, neither classification system of longitudinal/transverse/mixed nor OCS/OCV was predictive of SNHL, CHL, or FN palsy. A more robust database of audiologic results would be helpful in demonstrating this relationship.

Vein of Galen Aneurysmal Malformation Presenting As Persistent Pulmonary Hypertension in a Newborn: A Case Report.

We present a case of a newborn infant with a vein of Galen aneurysmal malformation (VOGM) who was admitted to our neonatal intensive care unit (NICU) with the diagnosis of persistent pulmonary hypertension of the newborn (PPHN). Further work-up at our institution, which included an echocardiogram and cranial ultrasound revealed VOGM. The patient was transferred to a children's center for further management of the vascular malformation where the patient subsequently died from high cardiac output heart failure. This study highlights the importance of considering a VOGM as a rare cause of PPHN in an infant.

Utility of skull radiographs in infants undergoing 3D head CT during evaluation for physical abuse.

BACKGROUND: Clinical practice guidelines recommend performing head CT and skull radiographs (SR) when evaluating infants for physical abuse. We compared the accuracy of 3-dimensional CT (3DCT) and SR for detecting skull fractures. METHODS: We reviewed children <12 months evaluated for physical abuse undergoing 3DCT and SR between January 2017 and December 2018. 3DCT and SR images were blindly read by 2 radiologists. Interrater reliability (IRR) was calculated. Diagnostic accuracy was compared using McNemar's test. RESULTS: 158 infants with a mean age of 5.0 months underwent 3DCT and SR. Consensus reading identified 46 fractures (29.1%) on 3DCT and 40 fractures (25.3%) on SR. IRR was higher for 3DCT (κ = 0.95) than for SR (=0.65). 11 fractures were identified on 3DCT but not SR. 5 fractures were identified on SR but not 3DCT. There was no difference in the diagnostic accuracy of 3DCT and SR (χ2 = 1.56, p = 0.211). CONCLUSIONS: We found no difference in the accuracy of 3DCT and SR for detecting skull fractures in infants. Because 3DCT has better IRR and evaluates for both bony and intracranial injuries it is superior to SR. Omitting SRs may be acceptable if a 3DCT is performed, and would reduce radiation exposure without compromising diagnostic accuracy.

Hereditary hemorrhagic telangiectasia and juvenile polyposis: an overlap of syndromes.

Hereditary hemorrhagic telangiectasia (HHT) (Osler-Weber-Rendu syndrome) is a syndrome characterized by multiorgan telangiectases and arteriovenous malformations. A subset of patients with a mutation in the MADH4 gene on chromosome 18 exhibits an overlapping syndrome of HHT and juvenile polyposis (JPS). We present one such family. Genetic testing is warranted when either HHT or JPS is diagnosed, as early recognition of this syndrome overlap allows appropriate management of these patients.

Presentation of an H-type tracheoesophageal fistula in an adolescent male with cystic fibrosis: A case report and review of literature.

Congenital TEFs without esophageal atresia are rare but may occur more frequently than previously documented in literature. Careful history is required to suspect the diagnoses, as most patients will present with coughing associated with solid or liquids, recurrent unexplained pulmonary infections and complaints with eating. Some patients may show signs of chronic airway changes from recurrent aspiration pneumonia at the time of presentation. Diagnosis is challenging, with multiple imaging modalities including x ray, CT scan and esophogram able to identify a fistula. Surgery is required to improve quality of life and prevent chronic airway changes, and most cases repaired have no complications.

Neuropsychological outcome following thalamic stroke in adolescence: an identical twin comparison.

Objective: Medial thalamic stroke in adults commonly results in severe learning and memory impairments and executive dysfunction, particularly during the acute phase. However, there is limited research on the cognitive recovery from thalamic stroke in physically healthy adolescents. This study aimed to fill this gap in the literature by utilizing a monozygotic twin control to investigate the neuropsychological outcomes of bilateral thalamic stroke in adolescence. Method: We evaluated an otherwise healthy 17-year-old male with a history of premature birth, developmental delay, and learning disability 2 and 7 months after he sustained a bilateral medial/anterior thalamic stroke of unknown etiology. His identical twin brother served as a case control. Results: The patient presented with improvements in many cognitive skills between assessments, most notably processing speed. Despite some mild improvement, however, he presented with significant deficits in fine motor speed/coordination, spatial perception, and rapid naming. Additionally, he exhibited persistent, severe deficits in verbal learning and memory. Relative sparing of executive functions (i.e., planning and set-shifting) and attention on standardized measures in this case may be explained by good underlying health, limited extra-thalamic damage, and/or recovery of function. The effects of thalamic injury resulted in minimal adaptive dysfunction or deterrence from academic or athletic success for the presented case. Conclusions: These results suggest risk for deficits in encoding of new verbal information following bilateral thalamic stroke in adolescence, as well as risk for persistent cognitive deficits despite initial improvements. This is consistent with descriptions of anterograde memory impairments in adults with similar lesions.

Diagnostic Potential of Neural Exosome Cargo as Biomarkers for Acute Brain Injury.

Objective: Neuronal exosomes purified from peripheral blood samples have been proposed as diagnostic tool in the setting of acute brain injury but never tested clinically. We hypothesized that exosome protein biomarkers would change over time following acute hypoxic brain injury and would predict response to therapy. Methods: Synaptopodin (SYNPO), an actin-associated protein present in postsynaptic spines, was evaluated as a potential biomarker as well as: synaptophysin, neuron-specific enolase, and mitochondrial cytochrome c oxidase. A secondary analysis was performed on neonatal samples collected at 8, 10, and 14 h after the initiation of therapeutic-controlled hypothermia for acute hypoxic-ischemic encephalopathy (n = 14). Neuronal exosomes were purified from serum and protein levels were quantified using standard ELISA methods. The primary study outcomes were length of stay (LOS), discharge on seizure medication (DCMED), and composite neuroimaging score (NIS). Results: The slope of change in neuronal exosome SYNPO between 8 and 14 h appeared to be the most promising biomarker for all three clinical study outcomes. SYNPO was highly correlated with LOS (-0.91, P < 0.001). SYNPO increased in 6/8 without DCMED and was worse or neutral in 5/5 with DCMED (P = 0.02). All four neonates with an abnormal NIS had neutral or decreasing SYNPO (P = 0.055). Other candidate biomarkers were not associated with outcomes. Interpretation: This report provides the first clinical evidence that neural exosomes turn over rapidly enough in the peripheral circulation to be used as a "troponin-like" test following acute brain injury. Optimal sampling and biomarkers likely vary with type of brain injury.

Childhood primary angiitis of the central nervous system with metachronous hemorrhagic infarcts: a postmortem study with clinicopathologic correlation.

This neuropathologic case study illustrates the discovery of metachronous hemorrhagic infarcts insinuating round mass-like lesions by magnetic resonance imaging in the setting of childhood primary angiitis of the central nervous system (cPACNS) raising diagnostic awareness of this unusual presentation in a clinical and neuroimaging context. The report underscores the importance of recurrent vasculitis-induced ischemic brain damage as a pathologic correlate of relapsing cPACNS and offers a critical reappraisal of common imitators as well as a clinicopathologic approach to differential diagnosis. Attention is drawn to the caveat that although magnetic resonance imaging findings at initial presentation may not be typical for stroke, they later exhibit attributes of cerebral infarction at both the subacute and chronic stages. A pattern of cPACNS characterized predominantly by multiple petechial-like cortical hemorrhages with pathologic features of hemorrhagic infarcts is recognized. The present study lends credence to the practice of a rigorous autopsy-based approach aimed at a better understanding of the anatomic pathology and biology of cPACNS and at facilitating prospective neuroimaging and biopsy-based surgical pathology correlations, ultimately enhancing diagnostic accuracy in clinical settings. Although PACNS is, by definition, a diagnosis of exclusion, it should be considered from the outset in the differential diagnosis of ischemic stroke or hemorrhagic stroke or of unusual and relapsing intra-axial mass-like CNS lesions in children, necessitating appropriate pathologic evaluation of brain biopsy specimens.