The NICU during COVID-19 Pandemic: Impact on Maternal Pediatric Medical Traumatic Stress (PMTS).

OBJECTIVE: The objective of this paper was to assess how hospital and outpatient clinic policies changes due to the coronavirus disease 2019 (COVID-19) pandemic impact pediatric medical traumatic stress (PMTS) symptoms in mothers of newborns admitted in a neonatal intensive care unit (NICU). STUDY DESIGN: Observational case-control study included the comparison between mothers of infants admitted in the NICU at birth during the COVID-19 pandemic and mothers of infants admitted in the NICU before the COVID-19 pandemic. The control group was selected matching 1:1 with the study group for the following infants' clinical variables: gender, type of pathology, gestational age, weight at birth, day of recovery, ventilator time days, and associated malformations. The Italian version of the Impact of Event Scale-Revised (IES-R) was used as a measure of PMTS. RESULT: Mothers of the study group (50) scored significantly higher than mothers of the control group on three of four scales of IES-R ("IES-R total": F = 6.70; p = 0.011; IES-R subscale "intrusion": F = 7.45; p = 0.008; IES-R subscale "avoidance": F = 8.15; p = 0.005). A significantly higher number of mothers in the study group scored above the IES-R total clinical cut-off compared with mothers of control group (72 vs. 48%; Chi2 = 6.00; p = 0.012). CONCLUSION: The COVID-19 pandemic acted as superimposed stress in mothers of newborns admitted in the NICU at birth determining high levels of PMTS. Clinicians and researchers should identify and implement novel strategies to provide family-centered care during the COVID-19 pandemic and beyond. KEY POINTS: · COVID-19 acted as superimposed stress on NICU population.. · PMTS in mothers got significantly worse during the COVID-19 pandemic.. · Alert on long-term consequences on child development..

Continence management in children with severe caudal regression syndrome: role of multidisciplinary team and long-term follow-up.

PURPOSE: Caudal regression syndrome (CRS) is a rare congenital abnormality including orthopedic deformities, urological, anorectal, and cardiac malformations. The clinical spectrum of CRS varies in severity, therefore multiple surgeries and complex medical care may be required with the efforts and support of a multidisciplinary team to ensure the most accurate treatment and the best outcome. The aim of our retrospective study was to evaluate the role of a multidisciplinary treatment and the long-term outcome in patients with severe CRS. METHODS: Clinical, surgical and psychological data were collected for all patients with diagnosis of CRS, treated at our Pediatric Hospital from January 1995 to December 2020. Patients with a severe form of CRS according to Pang's classification were included in the study. All patients have been followed by a multidisciplinary team composed by urologists, neurosurgeons, neonatal and digestive surgeons, orthopedic surgeon, physiatrists and psychologists. On admission, patients were screened for CRS with sacrum, spine and legs X-ray, spine MRI, renal ultrasound, voiding cystography and urodynamic, and renography. Clinical data about bowel function were evaluated. RESULTS: During the study period, 55 patients with CRS were treated at our Institution. Six out of 55 (10.9%), presented with severe form of CRS (5 pts with type 1; 1 pt, with type 2) and represent our study group. Diagnosis of severe CRS was made at birth because of the typical deformities of the pelvis (fusion of the iliac wings), and of the lower extremities (undeveloped legs with flexion of the knees, clubfoot). All patients presented with neurogenic bladder, 4/6 (66.6%) with vesicoureteral reflux (VUR) and 2/6 (33.3%) with renal agenesia and contralateral ectopic hypoplastic kidney. Bowel symptoms occurred in 5/6 pts (83.3%). All patients were started with an earlier clean intermittent catheterization (CIC) regimen. In 3 patients oxybutynin has been effective or well tolerated, while in other 3 onabotulinumtoxin A has been used. Vesicoureteral reimplantation has been performed in 1 patient, 2 required endoscopic treatment of VUR. On long-term, 2 patients required bladder augmentation with ileum and appendicostomy (Mitrofanoff). Low adherences in CIC have been observed in three patients, mainly at puberty. Two patients presented with chronic renal failure. One patient reported suicide proposal. Regarding social life, only one is regularly performing sport activity. CONCLUSION: CRS is characterized by maldevelopment of the caudal half of the body with variable involvement of the gastrointestinal, genitourinary, skeletal, and nervous systems. Management of CRS includes a wide variety of interventions to address the full spectrum of possible anatomical abnormalities. Hence, a multidisciplinary team is also mandatory for a correct bladder and bowel management, in order to maintain continence and preserve renal function, improve quality of life and increase self-esteem.

Anorectal malformation, urethral duplication, occult spinal dysraphism (ARM-UD-OSD): a challenging uncommon association.

PURPOSE: Urethral duplication (UD) is a rare malformation, which can be associated with other anomalies, like anorectal malformations (ARM). ARM has been described with occult spinal dysraphism (OSD). No ARM-UD-OSD combination has been reported. AIM: To share our experience and to discuss the management of ARM-UD-OSD association. METHODS: We retrospectively reviewed records of five boys with UD. Four of these had ARM-UD-OSD association. ARM was the first diagnosis in all; OSD and UD was detected during screening for associated malformation. RESULTS: All patients underwent ARM correction, 3 after colostomy. All reached fecal continence, 3 are performing bowel management. Three patients underwent UD surgical correction. Because of symptoms' worsening, 2 children had detethering surgery. At a mean follow-up of 9.5 years, all patients have normal renal function, 3 are on clean intermittent catheterization (CIC) for neurogenic bladder (1 has a cystostomy, another one an appendicostomy). CONCLUSIONS: UD and OSD should be considered in patients with ARM. Children with these conditions associated must be centralized in a third-level Center and management carefully planned; in particular, urethral reconstruction should be weighed, considering CIC could be required. Suspicion of neurogenic bladder must be present in OSD patient.

Validity and reliability of the Italian version of the cardiac quality of life questionnaire for pediatric patients with heart disease (PedsQLTM).

BACKGROUND: Congenital heart disease (CHD) accounts for nearly a third of all major congenital anomalies. Advances in pediatric cardiology shifted attention from mortality to morbidity and health-related quality of life (HRQOL) of patients with CHD and impact on their families. The purposes of this study were to assess the validity and reliability of the Italian version of the Pediatric Quality of Life (PedsQL) Cardiac Module and to create normative data for the Italian population. METHODS: This was an observational cross-sectional study of pediatric patients (aged 2-18 years) with congenital or acquired Heart Disease (HD) and their parents. Families were asked to complete the cardiac pediatric health-related quality of life questionnaire (the Italian PedsQL™ 3.0 Cardiac Module) and the generic pediatric health-related quality of life questionnaire (PedsQL™ 4.0 Generic Core Scales). The sequential validation procedure of the original United States version of the PedsQL™ 3.0 Cardiac Module was carried out under the instruction of the MAPI Research Institute. To assess construct validity, Pearson's correlation coefficients were assessed between scores on the Cardiac Module scales and scores on the scales of the General Module. To determine agreement between patient self-report and parent proxy-report, we used intraclass correlation coefficients (ICCs). To evaluate Internal consistency of items, we used Cronbach's alpha Coefficient. RESULTS: The study enrolled 400 patients. Construct validity is good between PedsQL Cardiac Module total scores and PedsQL total scores (p < 0.001). The recommended standard value of 0.7 was reached on the Cardiac and General Module core scales. Intercorrelations between PedsQL Cardiac module and PedsQL scores revealed medium to large correlations. In general, correlations between Patient self-reports are poorer than Parent-proxy ones. CONCLUSIONS: Cardiac PedsQL scores are valid and reliable for pediatric patients with congenital and acquired HD and may be useful for future research and clinical management.

Fixed the gap, solved the problem? Eating skills in esophageal atresia patients at 3 years.

Although eating problems have been described as long-term morbidities of esophageal atresia (EA), there have been few studies exploring eating outcomes in children born with EA as primary aim. Parents of children operated on for EA in our Institution from January 2012 to January 2016, answered a telephone structured interview developed specifically to conduct the present study, assessing eating skills at 3 years of age. Clinical data were collected from children's medical records. Parents (45 mothers and 6 fathers) of 51 children (male = 34; female = 17) with a median age of 3.5 years form the object of the study. Considering eating problems, parents reported that 23 children (45%) still have episodes of choking during meals at 3 years of age, 9 (45%) of these have more than one episode a week, and 19 parents (39%) reported higher levels of anxiety during mealtimes. Forty-four children (86%) were described by their parents as able to eat alone, 32 (65%) accepted all food textures and 45 (90%) was described as curious about food (3 years). Forty-three (86%) parents let their children eat with other people. Correlations showed that weaning age was significantly associated with number of dilatations (rs = 0.35, P = 0.012), days of mechanical ventilation (rs = 0.40, P < 0.001), and presence of gastrostomy tube at discharge (rs = 0.45, P < 0.001). Chewing age resulted associated with number of dilatations (rs = 0.34, P < 0.01) and days of mechanical ventilation (rs = 0.38, P < 0.01). Presence of choking episodes was associated with curiosity about food (rs = 0.29, P < 0.05), while frequent choking episodes were associated with higher parental anxiety during mealtimes (rs = 0.45, P < 0.05). In order to prevent delay in the achievement of eating developmental milestones in children operated on of EA, we advocate a dedicated preventive intervention from birth to follow-up.

Couples Facing the Birth of a Newborn with a Congenital Anomaly: PTSD Symptoms in the First Year.

OBJECTIVE: The objective of this study was to assess the presence of posttraumatic stress disorder (PTSD) symptoms in parental couples of newborn requiring early surgery at 6 and 12 months after birth. STUDY DESIGN: A longitudinal study was set up from January 2014 to June 2015. As a measure of PTSD, we used the Italian version of the Impact of Event Scale-Revised (IES-R). RESULTS: Thirty-four couples form the object of the study. At 6 months, half of mothers (52.9%) and fathers (44.1%) reported traumatic stress symptoms above the clinical cutoff. Percentages remained stable at 12 months. When parental gender and length of follow-up were compared with two-factor analysis of variance, none had an impact on IES-R score, nor an interaction between these factors was found. A significant correlation of IES-R total score was present within the couple both at 6 and 12 months (6 months-r: 0.6842, p < 0.0001 and 12 months-r: 0.4045, p = 0.0177). CONCLUSION: Having a child with a repaired malformation represents a complex prolonged stressful situation with persistent burden for both parents who are at high risk of developing PTSD symptoms.

Does Ventilatory Time Retain Its Validity in Predicting Neurodevelopmental Outcome at Two Years of Age in High-Risk Congenital Diaphragmatic Hernia Survivors?

Objective To evaluate if in high-risk non-extracorporeal membrane oxygenation (ECMO)-treated congenital diaphragmatic hernia (CDH) survivors, ventilatory time (VT) is correlated to and can be used as clinical marker of neurodevelopmental delay at 2 years of age. Study Design Cohort study was conducted between 2008 and 2012. Mental, motor, and language development were assessed by the Bayley Scales of Infant and Toddler Development III. The correlation between VT and neurodevelopmental outcome (NDO) was analyzed using Pearson's test. Receiver operating characteristic (ROC) analysis was performed to determine the accuracy and best cutoff value of VT to predict the risk of neurodevelopmental delay. Statistical significance was set at p < 0.05. Results A total of 49 patients form the subject of this study. VT during first admission was inversely correlated with cognitive (r = -0.4116; p = 0.0033), motor (r = -0.4241; p = 0.0024), and language development (r = -0.3564; p = 0.0119). Using ROC curve analysis, VT was a significant predictor for neurodevelopmental delay in the cognitive (area under the curve [AUC]: 0.864, sensitivity: 100; specificity: 66.67; p < 0.0001) and motor (AUC: 0.902; sensitivity: 100; specificity: 73.17; p < 0.0001) scales, but not in the language scale. The best cutoff value for both scales was 9 days. Conclusion Within a population of high-risk non-ECMO-treated CDH survivors, VT appears to retain its validity as a clinical marker of adverse NDO in cognitive and motor domains.

Continuous intravenous analgesia with fentanyl or morphine after gynecological surgery: a cohort study.

PURPOSE: This retrospective study aims to compare postoperative pain relief offered by continuous intravenous infusion of either fentanyl or morphine. METHODS: Sixty American Society of Anesthesiologists Physical Status I and II women who had undergone open gynecological surgery were enrolled. All patients received total intravenous postoperative analgesia for 24 h with continuous infusion of either fentanyl or morphine at comparable doses (38 patients received 0.3 µg/kg/h fentanyl and 22 received 0.02 mg/kg/h morphine). The primary endpoint was the need for analgesic rescue therapy during the postoperative period as assessed by an experienced nurse blinded to the design of the study, while the time to gastrointestinal bowel recovery was the main safety outcome measure. Visual analog scale was used to evaluate postoperative pain. Ramsay sedation score, multiparametric monitoring, bowel function and adverse effects were also recorded at 1, 6, 12, 18 and 24 h after the end of surgery. RESULTS: Data analysis showed that four patients (10 %) in the fentanyl group versus eight patients (36 %) in the morphine group needed to be treated with analgesic rescue drugs [unadjusted OR for fentanyl 0.2 (0.05-0.80); p = 0.02]. Patients treated with fentanyl showed a faster gastrointestinal recovery [1 (1-2) vs 3 (2.7-4) days; p < 0.001] and a shorter hospital length of stay [4 (3-5) vs 5.5 (5-7.2) days; p < 0.001]. CONCLUSION: In low-risk patients undergoing open gynecological surgery, continuous intravenous infusion of both fentanyl and morphine for postoperative pain relief is effective. In our cohort of patients, continuous intravenous infusion of fentanyl was associated with lower need for analgesic rescue drug, faster bowel recovery and shorter hospital length of stay.

Gershoni-Baruch syndrome: First report of a surviving child.

Gershoni-Baruch syndrome is an extremely rare malformation complex characterized by omphalocele, diaphragmatic hernia, radial ray defects, and cardiovascular abnormalities. Autosomal recessive inheritance was suggested. To date, academic literature described only seven patients fulfilling the diagnostic criteria for the condition. None survived the neonatal period. This study reports the first individual with Gershoni-Baruch syndrome surviving past early infancy.

Seeing Their Children in Pain: Symptoms of Posttraumatic Stress Disorder in Mothers of Children with an Anomaly Requiring Surgery at Birth.

Objective Assess the presence of posttraumatic stress disorder (PTSD) symptoms in mothers of newborns requiring early surgery. Study Design Mothers of newborns operated on for a congenital anomaly underwent a semi-structured interview on their experience 6 months postpartum. Interviews were audiotaped, transcribed verbatim, and analyzed for symptoms of the three major criteria of PTSD: re-experiencing, avoidance, and heightened arousal. Results A total of 120 mothers took part in the study; their children were affected by one of the following congenital anomaly: esophageal atresia (n = 29); congenital diaphragmatic hernia (n = 38); midgut malformations (n = 38); and abdominal wall defects (n = 15). Two mothers did not show any symptoms; 12 mothers (10%) had one posttraumatic symptom, 77 (64.2%) had two, and 29 (24.2%) had three. Overall, 106 mothers (88.4%) presented at least two symptoms. Conclusion PTSD can be considered a useful model to describe and comprehend mothers' reactions in this specific population. Preventive interventions and dedicated follow-up program should be offered to these families.

Predictive validity of the Italian parental questionnaire for developmental evaluation at age 4 (QS4-G).

AIM: To examine whether the results at 4 years of age of the developmental questionnaire QS4-G can predict the outcome of cognitive, neuropsychological and academic abilities 4-6 years later. The QS4-G is a validated parental questionnaire designed for the screening and surveillance of the neuropsychological and behavioural developmental status of 4-year-olds (93 questions). METHODS: Longitudinal prospective study on a subsample of the QS4-G validation original sample was conducted. According to previous results, the sample was divided into two groups: 'at risk' and 'not at risk'. Sensitivity, specificity, accuracy and likelihood ratios were assessed and referred to outcomes. RESULTS: Thirty-five children were classified as 'not at risk' and 16 as 'at risk'. There were significant associations between past QS4-G score and cognitive, neuropsychological and academic abilities 4-6 years later. With the same cut-off identified at the first cross-sectional study, sensitivity and specificity for difficulties in cognitive development were 90% and 83% while in the neuropsychological abilities 62% and 90%, respectively. A lower predictive validity was found for difficulties in academic abilities (sensitivity 43%, specificity 86%). QS4-G specific area scores showed significant correlations with related academic tests at follow-up (rho range: 0.404-0.565, P < 0.005). CONCLUSIONS: QS4-G shows good predictive validity for cognitive development and neuropsychological abilities. The risk of false negatives for academic abilities can be reduced by analysing the specific area results of QS4-G, which show good correlations with related tests at follow-up.

Surgical gastrointestinal anomalies including diaphragmatic hernia: Does type of anomaly affect neurodevelopmental outcome?

OBJECTIVE: To describe short-term neurodevelopmental outcome of infants operated on for congenital anomalies (CA) and assess the impact of type of CA on the outcome. STUDY DESIGN: From 2008 to 2010 newborns operated on for CA were enrolled in a cross-sectional follow-up study including three distinct groups: infants of 6 months (group A), infants of 12 months (group B), and children of 24 months (group C). Each group was divided into five subgroups: (1) esophageal atresia; (2) congenital diaphragmatic hernia; (3) midgut malformations; (4) abdominal wall defects; (5) colorectal malformations. Each group of patients underwent a neurodevelopmental evaluation with Bayley III. RESULTS: In all, 150, 156, and 84 babies were enrolled in groups A, B, and C, respectively. Mean (standard deviation) Mental Scale score was 94.65 (8.75), 98.76 (11.03), and 100.60 (12.04) in groups A, B, and C. Mean (standard deviation) Motor Scale score was 96.89 (11.62), 99.23 (14.83), and 103.60 (12.90) in groups A, B, and C. No significant differences were found among the five subgroups considered. CONCLUSION: Regardless of type of malformation, short-term neurodevelopmental outcome of children with gastrointestinal anomalies including diaphragmatic hernia falls within normal range, suggesting that neither being born with a CA nor its type is per se a risk factor for neurodevelopmental delay.

Holography-guided procedural planning for modifying Venus P-valve implantation technique in patients with left pulmonary artery stents: a case-series.

Background: Venus P-valve™ (Venus Medtech, Hangzhou, China) is a self-expandable bioprosthetic valve that can be transcatheter-implanted in native right ventricular outflow tract (RVOT) patients. Valve implantation is technically challenging. Due to the implantation technique, left pulmonary artery (LPA) stents represent a relative contraindication to Venus P-valve. In this case series, we describe our experience in implanting Venus P-valve in patients with previous LPA stents and the use of holographic models to facilitate procedural planning. Methods and results: From January to October 2023, 17 patients were scheduled for Venus P-Valve implantation. 16/17 (94%) patients were successfully implanted. 3/16 (18.7%) patients underwent Venus P-valve implantation with LPA stents. All patients underwent pre-operative CT scan. CT data set were employed to create three-dimensional (3D) holographic models (Artiness, Milan, Italy) of the entire heart, which were useful to plan valve implantation with a modified technique. Procedural success rate was 100%. No procedural complications occurred. All three patients presented good haemodynamic and angiographic results at discharge and follow-up visits. Conclusion: This case-series underscores the feasibility of Venus P-valve implantation in patients with previous LPA stents. The use of holographic models facilitated procedural planning in these challenging anatomical scenarios.

Flurbiprofen in the subglottic space to prevent postoperative sore throat after cardiac surgery: A randomized double-blind study.

STUDY OBJECTIVE: Postoperative sore throat (POST) and hoarseness are common complications of tracheal intubation. This study aims to evaluate the efficacy of flurbiprofen administered through the subglottic port of tracheal tubes to prevent POST after cardiac surgery. DESIGN: Single-center, prospective, randomized, double-blind, placebo-controlled trial. SETTING: Tertiary Care Referral University Hospital (Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome). PATIENTS: Included 71 patients undergoing for elective cardiac surgery. Inclusion criteria were (a) age between 50 and 75 years, (b) NYHA class I or II, (c) surgery for myocardial revascularization or valve repair or replacement under cardiopulmonary bypass. INTERVENTION: Patients were double blind randomized to receive flurbiprofen or saline in the subglottic port of the endotracheal tube (groups F and P). The solution was injected ten minutes after tracheal tube placement, ten minutes after ICU admission and ten minutes before tracheal tube removal. MEASUREMENTS: The primary outcome was to assess the effect of topical flurbiprofen administered through the subglottic port of the tracheal tube to prevent post-operative sore throat (POST). The secondary outcomes were the presence of hoarseness safety and patient's subjective satisfaction with their recovery. We did not report any exploratory outcomes. MAIN RESULTS: We analyzed 68 patients, 34 patients in each group. In group F, two patients complained of POST and hoarseness (5.9%), while all controls did. The two groups significantly differed in the severity scores (VAS and TPS for sore throat and HOAR for hoarseness) at all time points. In group P, patients reported mild to moderate symptoms that significantly improved or disappeared 36 h after tracheal tube removal. According to the multivariable model, hoarseness affected women less than men, in the control group (p = 0.002). None of the patients in either group reported any adverse effects. CONCLUSIONS: Repeated administration of flurbiprofen through the subglottic port of tracheal tubes reduced the incidence of sore throat and hoarseness after cardiac surgery without evidence of complications.

Cochlear Implantation in Intralabyrinthine Schwannoma: Case Series and Systematic Review of the Literature.

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature.

Corrigendum: Case report: Pragmatic impairment in multiple sclerosis after worsening of clinical symptoms.

[This corrects the article DOI: 10.3389/fpsyg.2022.1028814.].

Left atrial remodeling in hypertrophic cardiomyopathy and Fabry disease: A CMR-based head-to-head comparison and outcome analysis.

BACKGROUND: Hypertrophic cardiomyopathy (HCM) and Fabry disease cardiomyopathy (FD) are phenocopies, as they show left ventricular hypertrophy (LVH). The left atrium (LA) is emerging as a potential marker of disease severity in both cardiomyopathies. The present study compares HCM and FD cardiomyopathy with similar degree of LVH, exploring LA morpho-functional parameters and the correlates of clinical outcome. METHODS: We performed a comprehensive CMR-based comparison between 30 HCM and 30 FD patients matched on age, sex, BSA, LV mass and major cardiovascular risk factors affecting LA remodeling (arterial hypertension and diabetes). 30 healthy controls were also included. CMR feature tracking (CMR-FT) analysis, T1 mapping and conventional parameters were evaluated. Patients also underwent transthoracic echocardiography for LV diastolic function assessment. Clinical events at follow-up were collected (atrial and ventricular events, bradyarrhythmia, heart failure (HF) hospitalization and death). RESULTS: HCM patients showed greater LA remodeling compared to FD patients, namely higher LA end-systolic volume index (LAVi max), lower LA-ejection fraction (LA-EF) and worse reservoir (εs) and booster function (εa) (all p < 0.05). Accordingly, these parameters have demonstrated good potential for distinguishing between FD and HCM (AUC 0.68-0.73, all p < 0.05), with LAVi max being an independent predictor for HCM diagnosis (OR 1.07, 95%CI 1.011-1.132, p 0.02). Moreover, in HCM patients a significant association between εs and HF occurrence was observed at 2-year follow-up (OR 0.85, 95%CI 0.72-0.99, p 0.04). CONCLUSIONS: In HCM, LA remodeling is greater than in FD cardiomyopathy with similar LVH, and reservoir strain is associated with HF at follow-up.