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Background In congenital diaphragmatic hernia (CDH) survivors, failure to thrive is a well-known complication, ascribed to several factors. The impact of lung volume on growth of CDH survivors is poorly explored. Our aim was to evaluate if, in CDH survivors, lung volume (LV) after extubation correlates with growth at 12 and 24 months of life. Method s LV (measured as functional residual capacity-FRC) was evaluated by multibreath washout traces with an ultrasonic flowmeter and helium gas dilution technique, shortly after extubation. All CDH survivors are enrolled in a dedicated follow-up program. For the purpose of this study, we analyzed the correlation between FRC obtained shortly after extubation and anthropometric measurements at 12 and 24 months of age. We also compared growth between infants with normal lungs and those with hypoplasic lungs according to FRC values. A p < 0.05 was considered as statistically significant. Results We included in the study 22 CDH survivors who had FRC analyzed after extubation and auxological follow-up at 12 and 24 months of age. We found a significant correlation between FRC and weight Z-score at 12 months, weight Z-score at 24 months and height Z-score at 24 months. We also demonstrated that CDH infants with hypoplasic lungs had a significantly lower weight at 12 months and at 24 months and a significantly lower height at 24 months, when compared to infants with normal lungs. Conclusion We analyzed the predictive value of bedside measured lung volumes in a homogeneous cohort of CDH infants and demonstrated a significant correlation between FRC and growth at 12 and 24 months of age. An earlier identification of patients that will require an aggressive nutritional support (such as those with pulmonary hypoplasia) may help reducing the burden of failure to thrive. [ABSTRACT FROM AUTHOR]

Aim: To evaluate the correlation between sacral/vertebral anomalies and spinal cord anomalies (SCA) on MRI, in patients with anorectal malformation (ARM). Methods: Patients with ARM consecutively treated between January 1999 and August 2019 were included. Radiological imaging of sacrum and spine were retrospectively analyzed and correlated to the presence of SCA at MRI. Fisher's exact test and X2 test were used as appropriate; p<0.05 was considered statistically significant. Results: 348 patients with ARM were enrolled in the study, 147 presented SCA at MRI. 144 patients showed spinal bone anomalies, isolated vertebral and sacral anomalies were found in 17,6% and 35% respectively. Higher level of ARM was associated with a significant higher prevalence of sacral and vertebral anomalies. A significant correlation was found between the "level" of ARM and the presence of SCA (p<0.05). Sacral anomalies were significantly correlated with the presence of SCA at MRI (p<0.05). SCA were found in 70% of patients with vertebral anomalies (VA) and in 76% of patients with sacral anomalies. The presence of multiple malformations (vertebral and sacral anomalies) are strictly related to the presence of SCA. However, the absence of spinal bone anomalies does not exclude the presence of SCA. SD was the most represented type of SCA (n=94/147), of those 96% had fatty filum. Neurological or neurourological symptoms were detected in 11,5% patients (n=17) with SCA and required neurosurgical intervention. Conclusions: Our data confirm the strong relation between sacral or vertebral anomalies and SCA. However, in our series also patients without sacral/vertebral anomalies had SCA at MRI. Our results suggest that, despite the presence or absence of spinal anomalies, spinal cord MRI should be performed in all children with ARM, to allow a correct multidisciplinary follow-up and treatment. In fact, most patients with spinal bone and SCA are asymptomatic, but could develop clinical manifestations during their growth. [ABSTRACT FROM AUTHOR]

Pediatric medical traumatic stress (PMTS) is a psychological and physiological response of children and their families to pain, serious illness, and invasive medical procedures. We aimed to apply the PMTS model to parents of newborns operated at birth for a congenital malformation and to identify clinical and sociodemographic risk factors associated with PMTS symptoms at 6 months. We designed a cross-sectional study to assess PMTS symptoms (avoidance, arousal, reexperiencing) in parents of six months children operated on for a congenital anomaly, with the Italian version of the Impact of Event Scale – Revised (IES-R). One-hundred-seventy parents form the object of the study. Eighty-two parents (48.2%) fell over the clinical cut-off. Ventilatory time (p = 0.0001), length of hospital stay (p = 0.0001), associated anomalies (p = 0.0002), medical devices at discharge (p = 0.0001) and Bayley motor scale (p = 0.0002) were significantly correlated with IES-R Total and Subscale Scores. Multivariate linear regression showed length of hospital stay and number of associated anomalies as significant predictors of IES-R Scores. Regardless the type of anomaly and sociodemographic factors, it is the clinical history of the child which seems to predict the severity of PMTS symptoms in this population of parents. PMTS represents a useful model to describe the psychological reactions of parents of newborns operated at birth for a congenital malformation. NICU and outpatient pediatric staff should be aware of risk factors to identify families who may request early multidisciplinary interventions since the first admission. Prognosis study, level II. [ABSTRACT FROM AUTHOR]

Objectives: To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by the introduction of a new ultrasonographic (US) marker, defined as mediastinal shift angle (MSA) and to evaluate its ability in predicting postnatal survival at discharge.Methods: Twenty-four consecutive fetuses from singleton pregnancies with isolated left-sided CDH were included in the study group and then subdivided into group A (16 survivors) and group B (8 nonsurvivors). The study group was matched with a control group of 95 fetuses from singleton pregnancies free from structural and/or chromosomal anomalies. On the same US stored images commonly used for lung-to-head ratio (LHR) measurement, a landmark line was drawn from a point on the posterior face of the vertebral body, splitting it into two equal parts, to the mid-posterior surface of the sternum. Another landmark line was then traced from the same point of the vertebral body to touch tangentially the lateral wall of the right atrium. The angle between these two lines was used to quantify mediastinal shift and called "mediastinal shift angle" (MSA).Results: Median MSA was significantly different between group A (34.3° range 29.3-45.9°) and group B (42.7° range 34.1-58.9°) (p < .001) and between study group as a whole and the control group (19° range 13.8-25.9°) (p < .001). Statistical analysis confirmed an inverse correlation between MSA values and survival (p = .004). The best cutoff value for MSA was 43.7°, which demonstrated the highest discriminatory power (sensitivity 63%; specificity 93.75%).Conclusions: In fetuses with isolated CDH, the mediastinal shift may be quantified using mediastinal shift angle (MSA) and this US marker, similarly to the widely accepted and used US prenatal prognostic indicators (LHR and O/E LHR), seems to reliably predict survival. [ABSTRACT FROM AUTHOR]

The SIVI (Italian Society of Videosurgery in Infancy) guidelines are clinical practice guidelines edited and approved by the Society's steering committee. They are the products of a detailed systematic review of the literature, integrated with expert opinion in the field of pediatric minimally invasive surgery. These guidelines are intended to assist the pediatric surgeons experienced or not experienced in minimally invasive surgery, with the goal to inform choices related to the indications, approach, and techniques to use when treating the major pediatric surgical pathologies. Given the complexities of congenital malformations and other pediatric surgical conditions, as well as large variations in available regional health services, we must note that these guidelines are not intended as a cookbook recipe to follow for all possible patients. Rather, the guidelines should serve as a flexible framework, to be used by the physician in concert with the parents, to choose the best approach for each individual patient. Decisions tailored to available scientific knowledge and the needs and desires of the patient's family serve both patient autonomy and medical science. All guidelines are published in this scientific Journal, in order to ensure their availability to all physicians. The Guidelines project has been approved by the SIVI General Assembly of the 2016 Madrid congress. Review of guidelines has been performed by the Steering Committee of SIVI and experts. [ABSTRACT FROM AUTHOR]