Clinical Variables as Indicative Factors for Endoscopy in Adolescents with Esophageal Atresia.

INTRODUCTION: Gastro-esophageal reflux disease (GERD) occurs frequently in patients operated for esophageal atresia (EA). Longstanding esophagitis may lead to dysphagia, strictures, columnar metaplasia, and dysplasia with an increased risk of adenocarcinoma. Are clinical factors and non-invasive assessments reliable indicators for follow-up with endoscopy? MATERIAL AND METHOD: A follow-up study with inclusion of EA adolescents in Norway born between 1996 and 2002 was conducted. Clinical assessment with pH monitoring, endoscopy with biopsies, along with interviews and questionnaires regarding gastroesophageal reflux disease (GERD) and dysphagia were performed. RESULTS: We examined 68 EA adolescents. 62% reported GERD by interview, 22% by questionnaire. 85% reported dysphagia by interview, 71% by questionnaire. 24-hour pH monitoring detected pathological reflux index (RI) (>7%) in 7/59 (12%). By endoscopy with biopsy 62 (92%) had histologic esophagitis, of whom 3 (4%) had severe esophagitis. Gastric metaplasia was diagnosed in twelve (18%) adolescents, intestinal metaplasia in only one (1.5%). None had dysplasia or carcinoma. Dysphagia and GERD were statistically correlated to esophagitis and metaplasia, but none of the questionnaires or interviews alone were good screening instruments with high combined sensitivity and specificity. A compound variable made by simply taking the mean of rescaled RI and dysphagia by interview showed to be the best predictor of metaplasia (85% sensitivity, 67% specificity). CONCLUSION: The questionnaires and interviews used in the present study were not good screening instruments alone. However, combining dysphagia score by interview and RI may be helpful in assessing which patients need endoscopy with biopsy at each individual follow-up examination. LEVEL OF EVIDENCE: Level II prognostic study.

New insights in the prevalence of scoliosis and musculoskeletal asymmetries in adolescents with esophageal atresia.

BACKGROUND: Increased risk of scoliosis and musculoskeletal abnormalities in adolescents with esophageal atresia (EA) is reported, but the impact of these abnormalities on physical fitness and motor skills are not known. METHODS: Scoliosis was assessed radiographically and shoulder and chest abnormalities by a standardized protocol. Physical fitness was evaluated with Grippit, Six-minute walk test, and International Physical Activity Questionnaire and motor skills by Motor Assessment Battery for Children. RESULTS: Sixty-seven EA adolescents median 16 (13-20) years participated. The prevalence of significant scoliosis (≥ 20º) was 12% (8/67) whereas 22% (15/67) had mild scoliosis (10-19º). Vertebral anomalies occurred in 18/67 (27%), eight of them (44%) had scoliosis. The majority of adolescents (15/23) with scoliosis did not have vertebral anomalies. Musculoskeletal abnormalities were detected in 22-78%. Balance problems occurred three times more frequently than expected (44% vs. 15%, p = 0.004). Submaximal exercise capacity was significantly reduced compared to reference values (p < 0.001). Scoliosis ≥ 20º was related to reduced physical activity (p = 0.008), and musculoskeletal abnormalities to reduced physical activity and impaired motor skills (p = 0.042 and p < 0.038, respectively). CONCLUSIONS: Significant scoliosis was diagnosed in 12% of the EA adolescents and related to reduced physical activity. Musculoskeletal abnormalities identified in more than half of the patients, were related to reduced physical activity and impaired motor skills, and exercise capacity was significantly below reference group. EA patients with and without vertebral anomalies need health-promoting guidance to prevent impaired motor skills and consequences of reduced physical activity. LEVEL OF EVIDENCE: Prognostic Study, Level II.

Development and Validation of a Deep Learning Method to Predict Cerebral Palsy From Spontaneous Movements in Infants at High Risk.

Importance: Early identification of cerebral palsy (CP) is important for early intervention, yet expert-based assessments do not permit widespread use, and conventional machine learning alternatives lack validity. Objective: To develop and assess the external validity of a novel deep learning-based method to predict CP based on videos of infants' spontaneous movements at 9 to 18 weeks' corrected age. Design, Setting, and Participants: This prognostic study of a deep learning-based method to predict CP at a corrected age of 12 to 89 months involved 557 infants with a high risk of perinatal brain injury who were enrolled in previous studies conducted at 13 hospitals in Belgium, India, Norway, and the US between September 10, 2001, and October 25, 2018. Analysis was performed between February 11, 2020, and September 23, 2021. Included infants had available video recorded during the fidgety movement period from 9 to 18 weeks' corrected age, available classifications of fidgety movements ascertained by the general movement assessment (GMA) tool, and available data on CP status at 12 months' corrected age or older. A total of 418 infants (75.0%) were randomly assigned to the model development (training and internal validation) sample, and 139 (25.0%) were randomly assigned to the external validation sample (1 test set). Exposure: Video recording of spontaneous movements. Main Outcomes and Measures: The primary outcome was prediction of CP. Deep learning-based prediction of CP was performed automatically from a single video. Secondary outcomes included prediction of associated functional level and CP subtype. Sensitivity, specificity, positive and negative predictive values, and accuracy were assessed. Results: Among 557 infants (310 [55.7%] male), the median (IQR) corrected age was 12 (11-13) weeks at assessment, and 84 infants (15.1%) were diagnosed with CP at a mean (SD) age of 3.4 (1.7) years. Data on race and ethnicity were not reported because previous studies (from which the infant samples were derived) used different study protocols with inconsistent collection of these data. On external validation, the deep learning-based CP prediction method had sensitivity of 71.4% (95% CI, 47.8%-88.7%), specificity of 94.1% (95% CI, 88.2%-97.6%), positive predictive value of 68.2% (95% CI, 45.1%-86.1%), and negative predictive value of 94.9% (95% CI, 89.2%-98.1%). In comparison, the GMA tool had sensitivity of 70.0% (95% CI, 45.7%-88.1%), specificity of 88.7% (95% CI, 81.5%-93.8%), positive predictive value of 51.9% (95% CI, 32.0%-71.3%), and negative predictive value of 94.4% (95% CI, 88.3%-97.9%). The deep learning method achieved higher accuracy than the conventional machine learning method (90.6% [95% CI, 84.5%-94.9%] vs 72.7% [95% CI, 64.5%-79.9%]; P < .001), but no significant improvement in accuracy was observed compared with the GMA tool (85.9%; 95% CI, 78.9%-91.3%; P = .11). The deep learning prediction model had higher sensitivity among infants with nonambulatory CP (100%; 95% CI, 63.1%-100%) vs ambulatory CP (58.3%; 95% CI, 27.7%-84.8%; P = .02) and spastic bilateral CP (92.3%; 95% CI, 64.0%-99.8%) vs spastic unilateral CP (42.9%; 95% CI, 9.9%-81.6%; P < .001). Conclusions and Relevance: In this prognostic study, a deep learning-based method for predicting CP at 9 to 18 weeks' corrected age had predictive accuracy on external validation, which suggests possible avenues for using deep learning-based software to provide objective early detection of CP in clinical settings.

Impaired motor performance in adolescents with esophageal atresia.

AIMS: The study prospectively assessed motor development from infancy to adolescence in patients with esophageal atresia (EA). METHODS: At one year of age motor performance was evaluated with the Psychomotor Developmental Index (PDI) of the Bayley Scales of Infant Development, Second Edition (BSID-II), and as adolescents reevaluated with Motor Assessment Battery for Children, Second Edition (MABC-2). Associations to clinical factors were assessed. RESULTS: 23 EA patients were followed from infancy to adolescence. The median total PDI score in infancy was 102 (56-118) and the corresponding mean z-score was -0.006 (SD 0.995) and not significantly different from the reference values (p = 0.48). The median total MABC-2 score in adolescence was 75 (32-93) and the corresponding mean z-score -0.43 (SD 0.998) which is significantly below normal (p = 0.03). Children with impaired motor function in adolescence underwent significantly more rethoracotomies than those with normal motor performance (p = 0.037); whereas the two groups did not differ with respect to other clinical characteristics. CONCLUSION: From infancy to adolescence the motor performance in the group of EA patients deteriorated from within normal range to significantly impaired compared to reference values. Interdisciplinary follow-up programs from infancy to adolescence with close monitoring for motor function is necessary to detect motor impairments.

The Predictive Accuracy of the General Movement Assessment for Cerebral Palsy: A Prospective, Observational Study of High-Risk Infants in a Clinical Follow-Up Setting.

BACKGROUND: Early prediction of cerebral palsy (CP) using the General Movement Assessment (GMA) during the fidgety movements (FM) period has been recommended as standard of care in high-risk infants. The aim of this study was to determine the accuracy of GMA, alone or in combination with neonatal imaging, in predicting cerebral palsy (CP). METHODS: Infants with increased risk of perinatal brain injury were prospectively enrolled from 2009-2014 in this multi-center, observational study. FM were classified by two certified GMA observers blinded to the clinical history. Abnormal GMA was defined as absent or sporadic FM. CP-status was determined by clinicians unaware of GMA results. RESULTS: Of 450 infants enrolled, 405 had scorable video and follow-up data until at least 18-24 months. CP was confirmed in 42 (10.4%) children at mean age 3 years 1 month. Sensitivity, specificity, positive and negative predictive values, and accuracy of absent/sporadic FM for CP were 76.2, 82.4, 33.3, 96.8, and 81.7%, respectively. Only three (8.1%) of 37 infants with sporadic FM developed CP. The highest accuracy (95.3%) was achieved by a combination of absent FM and abnormal neonatal imaging. CONCLUSION: In infants with a broad range of neonatal risk factors, accuracy of early CP prediction was lower for GMA than previously reported but increased when combined with neonatal imaging. Sporadic FM did not predict CP in this study.

A change in temporal organization of fidgety movements during the fidgety movement period is common among high risk infants.

AIM: General movement assessment (GMA) at 9-20 weeks post-term, can effectively predict cerebral palsy. Our aim was to evaluate intra-individual variability of the temporal organization of fidgety movements (FMs) in high risk infants. MATERIAL AND METHODS: 104 High risk infants (66 males) with at least two video recordings from the FMs period participated. 45 of the infants had GA <28 weeks and/or BW ≤800 g. Mean post-term age at first and second assessments was 11.0 (8-16) and 14.0 (11-17) weeks, respectively, and median time-difference between the assessments was 2.0 (range: three days to six weeks) weeks. Video recordings were analyzed according to Prechtl's GMA. RESULTS: 33 (32%) Infants were classified differently at first and second assessments. Six infants (6%) changed from normal to abnormal, and 10 (10%) changed from abnormal to normal FMs. Seven of the ten who changed classification from abnormal to normal were born before GA 26 weeks. A change between intermittent and continual, which are both considered normal, was observed in 17 (16%) infants. CONCLUSION: A change in temporal organization of FMs is common in high risk infants. Especially in extremely preterm infants with abnormal FMs, more than one assessment should be performed before long-term prognosis is considered.

High prevalence of abnormal motor repertoire at 3 months corrected age in extremely preterm infants.

AIMS: To compare early motor repertoire between extremely preterm and term-born infants. An association between the motor repertoire and gestational age and birth weight was explored in extremely preterm infants without severe ultrasound abnormalities. METHODS: In a multicentre study, the early motor repertoire of 82 infants born extremely preterm (ELGAN:<28 weeks) and/or with extremely low birth weight (ELBW:<1000 g) and 87 term-born infants were assessed by the "Assessment of Motor Repertoire - 2 to 5 Months" (AMR) which is part of Prechtl's "General Movement Assessment", at 12 weeks post-term age. Fidgety movements were classified as normal if present and abnormal if absent, sporadic or exaggerated. Concurrent motor repertoire was classified as normal if smooth and fluent and abnormal if monotonous, stiff, jerky and/or predominantly fast or slow. RESULTS: Eight-teen ELBW/ELGAN infants had abnormal fidgety movements (8 absent, 7 sporadic and 3 exaggerated fidgety movements) compared with 2 control infants (OR:12.0; 95%CI:2.7-53.4) and 46 ELBW/ELGAN infants had abnormal concurrent motor repertoire compared with 17 control infants (OR:5.3; 95%CI:2.6-10.5). Almost all detailed aspects of the AMR differed between the groups. Results were the same when three infants with severe ultrasound abnormalities were excluded. In the remaining ELBW/ELGAN infants, there was no association between motor repertoire and gestational age or birth weight. CONCLUSION: ELBW/ELGAN infants had poorer quality of early motor repertoire than term-born infants.The findings were not explained by severe abnormalities on neonatal ultrasound scans and were not correlated to the degree of prematurity. The consequences of these abnormal movement patterns remain to be seen in future follow-up studies.