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Aim of the study: Lymphatic malformations (LMs) are rare entities, sometimes difficult to treat, that may be life-threatening when intricately connected to airway structures. Invasive treatments are occasionally required, with sclerotherapy considered the treatment of choice and surgery as a second-line approach. The aim of the present study was to evaluate our multidisciplinary team experience in treating newborns affected by LMs requiring operative management, while defining early outcomes.Methods: Retrospective review of all consecutive patients admitted for LMs requiring operative management between January 2000 and January 2019. Patients were mainly characterized based on anatomical district of the LM (and further stratified based on the development of respiratory distress), need for tracheostomy, number of sclerotherapies, indication for surgery, and residual disease beyond the 1st year. Morbidity and mortality were also evaluated. Fisher exact test and Mann–Whitney test were used as appropriate. Statistical significance was set at p < 0.05.Results: Fifty-seven patients were included in the study, 36 with cervicofacial and/or mediastinal LMs and 21 with LMs of other anatomical districts. Due to the risk of developing respiratory distress at birth, patients with cervicofacial and/or mediastinal LMs were divided into two groups (8/36 group A vs. 28/36 group B). Group A patients are at higher risk for tracheostomy (7/8 group A vs. 1/28 group B, p = 0.0001) and more often require surgical reduction of the residual lymphatic abnormality (5/8 group A vs. 4/28 group B, p = 0.013). They also require sclerotherapies more often, but the difference is not statistically significant (8/8 group A vs. 19/28 group B, p = 0.15). Patients with cervicofacial/mediastinal LMs frequently suffer from persistent residual disease beyond the 1st year of life, significantly more often in group A (7/8 group A vs. 12/28 group B, p = 0.043).Conclusion: LMs are rare conditions with potential life-threatening behavior. Their intrinsic clinical complexity requires a multidisciplinary approach to the affected patients. Planning a long-term follow-up is essential because of the late-term problems those patients may experience.

Background Late-presenting congenital diaphragmatic hernia (L-CDH) diagnosis is a challenge for its clinical various presentation. In literature radiologic misdiagnosis is up to 62%. The aim of this study is analyze clinical findings about our cases series in a particular setting of Pediatric Emergency Department (PED) and review of literature. Methods We retrospectively analyzed the medical records of children older than 1 month, operated for L-CDH from November 2009 to December 2019 presented to PED, with a total of 20 children (5 Morgagni and 15 Bochdaleck). Results The median age at diagnosis was 1,48 years. 50% patients had a history of previouses symtptoms with a mean duration of 243,75 days. In 30 % of cases associated anomalies were presented, mainly cardiovascular. 25% of patients had undergone to chest X-ray in past history with misdiagnosis of pneumonia, microganulia and broncovascular thickening. Misdiagnosis are manly among left sided Bochdaleck hernia. Respiratory distress alone or associated with vomiting is commonest acute symptom refered to PED. Respiratory symptoms were more common in younger children, while gastrointestinal ones in older children (not statistically differences p=0,8769). Post-operative recurrence were frequent only in cases of right sided CDH and comparing with left ones, there is a difference statistically significant (p=0.0476). Conclusions L-CDH should be suspected in cases of unexplained acute respiratory distress and vomiting, particulary when children are affected to associate cardiovascular malformation. In cases of patients with long previouses symptoms and various accesses to PED is important to emphasize history of respiratory distress, dysphagia and failure to thrive and should be considered a chest X-ray.

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. Methods 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.

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 (M...

Congenital diaphragmatic hernia (CDH) survivors present long-term morbidities in several systems, including the neurodevelopmental, gastrointestinal, pulmonary, and musculoskeletal ones, and CDH long-term sequelae are increasingly being recognized. Due to high co-morbidity, health related quality of life in a significant proportion of CDH patients might be compromised. As a consequence of consciousness on the long-term sequelae of CDH survivors, and their consequences for life, several follow-up programs were brought to life worldwide. In this review, we will summarize the long-term sequelae of CDH survivors, the impact of new treatments, and analyze the consistency of follow-up programs.

Background Oesophageal atresia (OA) is characterized by a significant morbidity during the neonatal period. Affected infants are exposed to multiple potentially nephrotoxic factors, predisposing them to acute kidney injury (AKI), which is associated with poorer outcomes. Children who survive neonatal AKI are at risk of long-lasting renal complications (including chronic kidney disease and hypertension). Aims The purpose of this study is to investigate AKI prevalence in infants with OA and identify potential contributing factors. Methods Retrospective analysis was conducted on clinical records of patients with OA between 2017 and 2018 in a single referral centre. AKI severity was defined according to established international pRIFLE criteria (Risk of renal dysfunction, kidney Injury, Failure or Loss of kidney function and End-stage renal disease) based on change in serum creatinine and urine output during hospitalization collected 5 days after birth. Risk factors, (including potentially nephrotoxic medications, significant infection, urine output, blood pressure), and outcome data (length of hospitalization, duration of intubation) were recorded. Statistical analysis was performed using Graphpad. Results Thirty-nine OA cases were identified; 6 were excluded for lack of data and 33 were included in the study group. Eighteen (55%), who developed AKI (pRIFLE category Risk or Injury), were compared with 15 patients (45%) with no AKI. AKI was significantly higher in infants with long-gap OA (P = 0.02), significantly associated with vancomycin administration (P = 0.03), with the use of three or more antibiotics during hospitalization (P = 0.0005), and with postoperative muscle paralysis (P = 0.001). Infants with OA and AKI experienced longer mechanical ventilation (P = 0.004) and hospital stay (P = 0.003). No significant association was found with hypotension, or steroid exposure. Incomplete recording of urine output prevented stratification of AKI based on this parameter. Conclusions OA, particularly more severe cases, are at risk of AKI. Renal function should be routinely assessed, during hospitalization and follow-up. Focusing on prevention and avoiding prolonged courses of nephrotoxic medications may improve outcome.

Background Esophageal atresia (EA) prognosis have improved significantly over the past three decades. Research and clinical attention has shifted to neurodevelopmental outcomes and quality of life. Aim The aim of this study wasto examine neurodevelopmental outcomes and to identify clinical and sociodemographic risk factors in a cohort of infants with EA. Methods An observational prospective longitudinal study was conducted between 2009 and 2017. Neurodevelopment was assessed at 6 and 12 months by Bayley Scales of Infants and Toddler Development—3rd Edition. Clinical and sociodemographic variables included were gender, birthweight, gestational age, associated malformations, number of hospitalizations, surgeries and dilatations at 12 months, days of mechanical ventilation, parental age, education level, and socioeconomic status. Results Ninety-six infants were enrolled in the study at 6 months and 73 of them were evaluated also at 12 months. Analysis showed significant differences between motor development at 6 and 12 months (M6 = 95.39, SD = 15.71; M12 = 91.83, SD = 12.87; t = 0.245, P = 0.017); significant differences emerged also between cognitive development at 6 and 12 months (M6 = 91.80, SD = 11.70; M12 = 100.92, SD = 15.39; t = −5.10, p = .000). Infants with long-gap AE achieved the worst scores in cognitive (r = -.28, P Conclusions Infants operated on for AE are at risk of neurodevelopmental impairment in the first year of life. Findings support the association between neurodevelopmental outcomes and clinical risk factors. Careful interdisciplinary follow-up is essential for early detection of neurodevelopmental delay.

Introduction Since recently, after detection of eosinophilic predominant inflammation of esophagus a trial with proton-pump inhibitors (PPIs) was needed to individuate children with PPI-responsive esophageal eosinophilia (PPI-REE), only those non-responders received eosinophilic esophagitis (EoE) diagnosis. In 2018 updated international consensus suggested removing the PPI trial as a diagnostic criterion and consider PPIs as a treatment together with diets and topical steroids. The role of PPIs is evaluated in children with esophageal atresia (EA) and EoE versus EoE from general population. Method A retrospective chart review of both children with EA and EoE followed-up from at January 2005 has been performed. According to ESPGHAN guidelines published in 2014 patients showing eosinophilic inflammation received high-dose PPI trial to identified PPI-REE. Those non-responders were labeled as EoE and underwent to dietary and/or topical steroid treatment. Demographics and disease characteristics of EA patients with EoE were analyzed and compared with those with EoE from general population. Results Overall, 370 EA and 118 EoE patients were analyzed. Of them 15 EA-EoE patients were detected. Consequently, in our cohorts, 4.0% of EA patients developed EoE. Male-to-female prevalence ratio was of 2.55 with no difference in gender prevalence between groups. At diagnosis EoE-EA children were significantly younger compared to EoE group (mean: 5.1 vs 10.8 years; P Conclusion Similar gender distribution and high prevalence of allergy suggest that common genetic susceptibility factors for EoE exist. However, high prevalence of PPI-REE coupled early EoE onset might also suggest that other factors (e.g. esophageal motility disorders) might play a physiopathological role in EoE development in EA children. Our study suggests that a stepwise approach with PPIs as a first-line treatment for EoE management in EA children should still be considered.

"Apple peel" intestinal atresia is a rare form of small bowel atresia, in which the duodenum or proximal jejunum ends in a blind pouch and the distal small bowel wraps around its vascular supply, in a spiral resembling an apple peel. The etiology of "apple peel" intestinal atresia is presently unknown, although a congenital or acquired intestinal vascular accident can have a role in the pathogenesis. We report a family in which the proband affected by "apple peel" intestinal atresia, had a sibling (an interrupted pregnancy), and a paternal cousin with cardiac left-sided obstructive lesions. Molecular testing for NOTCH1 gene was carried out in the proband, because pathogenic mutations in this gene have been associated with familial and sporadic cardiac left-sided obstructive lesions and vascular anomalies, both isolated or within the spectrum of the Adams-Oliver syndrome (AOS). The heterozygous c.2734C>T (p.Arg912Trp) NOTCH1 variant was found in the proband with "apple peel" intestinal atresia and in his father. This result argues for a possible causal relationship between NOTCH1 gene mutations and some forms of intestinal defects, through a vascular mechanism. The spectrum of NOTCH1-associated malformations is widened. Genetic counseling should take into account intrafamilial variable clinical expression and incomplete penetrance.

Aim Esophageal atresia (EA) and tracheoesophageal fistula (TEF) consist of a spectrum of rare congenital abnormalities. Although EA surgical treatment is well established, the outcome of EA with proximal fistula (type B and D EA) or isolated H-type fistula (type E EA) is poorly explored. These forms of EA shared a common surgical step: the need of a cervical approach to close the fistula. Therefore, the aim of present study is to evaluate postoperative outcomes of patients treated for Gross type B–D and E EA, on regards of their cervical surgery. Materials and methods A retrospective case series analysis of all patients affected by type B–D and E EA, and admitted to our tertiary care center between January 2003 and December 2014 was performed. All patients underwent preoperative flexible laryngo-tracheobronchoscopy (LTBS) as part of our standardized preoperative diagnostic assessment to define the diagnosis, evaluate preoperative vocal cord motility and to cannulate the fistula when required. Fistula closure was always performed through a right cervical access. Analysis of all cases and comparison between type B–D and E EA were performed. Mann–Whitney test, Chi-squared test and unpaired t test were used as appropriate; p Results During the study period, 180 EA newborns were treated. Proximal or isolated TEF was found in 18 patients (10%): 7 type B, 11 type E EA. Patients affected by type B and E EA/TEF frequently present associated major malformations (27%), and major cardiac abnormalities (44%). Major postoperative complications were: vocal cord paralysis (5 patients), bilateral in 2 infants requiring tracheostomy, cerebral ischemia (1 patient), and cardiac failure (1 patient). Conclusion Patients affected by type B and E EA have a high rate of associated abnormalities, and risk of possible sequelae. Postoperative complications are common, with possible transient vocal cord dismotility, but in some cases persistent paralysis may require tracheostomy. Therefore, both preoperative and postoperative LTBS is highly recommended to evaluate the presence of a proximal fistula, and vocal cord motility, even in asymptomatic patients, to rule out any possible intraoperative "surprise" and any vocal cord abnormality and to possibly define its pathogenesis (congenital vs. iatrogenic).

SummaryIntroduction : Patients operated on for congenital pulmonary malformations (CPM) have excellent survival rates, but little is known about long-term morbidity. Our aim is to report the sequelae in patients operated on for CPM in infancy and to define factors that may influence their outcome. Methods: All patients operated on for major congenital anomalies are followed in a dedicated outpatient program and evaluated at 6, 24, and 48 months of life (corrected for gestational age) and at school age at 4, 6, 8, and 12 years of life. The data are prospectively collected. Patients operated on for CPM and enrolled in the follow-up clinic between January 2004 and December 2010 are compared with a control group of term infants operated on for inguinal hernia, without other major congenital or acquired abnormalities. The two groups were compared for auxological, respiratory, and orthopedic outcome. Results: In the study period, 76 consecutive patients with CPM attended our dedicated follow-up clinic. Eight non-operated patients were excluded from the study. Age at follow-up was 82.0 (56.1–103.7) months in CPM patients and 83.5 (75.2–90.4) months in controls (P = 0.79). Fifty-three patients with CPM (78%) had one or more clinical or radiological abnormality versus six (16%) control patients (OR [95%CI] 16.5 [5.8–47.2]; P

Background: A high prevalence (9.5–30%) of eosinophilic esophagitis (EoE) in patients with esophageal atresia (EA) has been reported. The application of the EoE criteria to EA patients might be problematic. To date, only studies using a “routine” biopsy approach, even in asymptomatic patients, have been performed. The aim of the study was to establish the prevalence of EoE among symptomatic EA patients (EA/EoE group) without anastomotic stricture (AS) and to compare their characteristics with those of EoE patients from general population (EoE group). Methods: From 2005 to 2018, we reviewed charts of children with EA and EoE. “Selective” biopsy approach only in EA children without AS and/or endoscopic feature of EoE was performed. Characteristics of EA/EoE and EoE groups were compared. Results: Among 370 EA and 118 EoE, 15 EA/EoE patients were detected (4.0% of EA patients). Male predominance and a high prevalence of allergy without differences between EA/EoE and EoE groups was observed. EA/EoE children were significantly younger (p

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.

Summary Data on the neurodevelopmental outcome of esophageal atresia (EA) survivors are scarce, controversial, and based on small samples. This is an observational prospective longitudinal study on a selected cohort of low-risk EA survivors. We considered a low-risk EA survivor a patient with the following characteristics: gestational age >32 weeks, no long gap, no genetic or chromosomic anomaly associated with neurodevelopmental delay, and no further major surgical congenital anomalies. Infants were evaluated with scales derived from the Bayley Scales of Infant and Toddler Development – 3rd Edition at 6 and 12 months, with a score of 100 considered normal for each scale. Analysis of variance was used to assess differences of cognitive and motor development. Linear regression was used to assess the impact of the following clinical and sociodemographic variables: gender, birthweight, gestational age, length of hospital stay, number of surgeries and number of esophageal dilatations during first hospitalization, days of mechanical ventilation, weight at follow up, number of surgeries and esophageal dilatations at follow up, parental age, educational level, and socioeconomic status. Thirty children form the object of the study. The mean (standard deviation [SD]) cognitive scale's score was 93.7 (7.5) and 98.2 (9.6) at 6 and 12 months, respectively (P 5° percentile: 88.8 (6.3) and 100.5 (8.9), respectively. At 12 months, children with unemployed mothers had a mean (SD) motor score significantly lower when compared with those in the other socioeconomic classes: 87.7 (9.8) and 100.6 (12.4), respectively. In conclusion, parents of babies operated on for low-risk EA can be reassured about neurodevelopmental outcome at least up to 1 year of age. When offering a multidisciplinary follow-up program, underweight patients should deserve particular attention to promote their quality of life and support their global development.

PURPOSE The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH. METHODS All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac. RESULTS A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p

Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal reconstruction include the use of native esophagus or esophageal replacement with stomach, colon, or small intestine. Nonetheless, there is a consensus among most pediatric surgeons that the preservation of the native esophagus is associated with better postoperative outcomes. Thus, every effort should be made to conserve the native esophagus. The present study is aimed at critically reporting our experience focused on a standardized protocol based on the preoperative assessment of the gap in all cases and reviewing the present literature because no consensus is available regarding many aspects of LGEA (from definition to treatment). All newborn infants treated since 1995 for esophageal atresia (EA), regardless of type, were included in the present study. Identification of LGEA patients (gap ≥3 vertebral bodies) was performed based on preoperative esophageal gap measurement. The selected patients were grouped based on EA type (A/B vs. C/D) and whether they were referred from an outside institution or not. Postoperative outcome was compared. Statistical analysis was performed with the Fisher's exact test and Mann-Whitney test as appropriate, with P < 0.05 considered statistically significant. Two hundred and nineteen patients have been consecutively treated between 1995 and 2012 with the following EA subtypes: type: A 25 (11.4%); B 6 (2.7%); C 182 (83.1%); D 3 (1.4%); E 3 (1.4%). Fifty-seven patients (26%) were classified as LGEA: type A-B, 31 (54.4%); type C-D, 26 (45.6%). Twenty seven (47%) of these patients were referred after at least one failed attempt at esophageal correction: type A-B, 15 (55%); type C-D, 12 (45%). Only one patient ultimately required esophageal substitution, with an overall survival rate of 94%. A standardized perioperative protocol enhances the possibility of preserving the native esophagus in cases of LGEA. Gap measurement can be accurately defined before surgery in all patients with EA. Esophageal anastomosis (either immediate or delayed repair) is almost always feasible; esophageal substitution should only be considered after a rigorous attempt at achieving end-to-end esophageal anastomosis.

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.

Background: Since mortality in congenital diaphragmatic hernia (CDH) is decreasing, morbidity such as neurodevelopmental outcome is becoming increasingly important. Objectives: We evaluated neurodevelopmental outcome in high-risk CDH patients treated according to the CDH EURO Consortium standardized treatment protocol. Methods: This observational, prospective cohort study was conducted in two European centers. Neurodevelopment of 88 patients (Rotterdam n = 49; Rome n = 39) was assessed at 12 and 24 months with the Bayley Scales of Infant Development (BSID)-II-NL (Rotterdam) or BSID-III (Rome). Data of the centers were analyzed separately. Results: Cognition was normal in 77.8% of children from Rotterdam and in 94.8% from Rome at 12 months, and in 70.7 and 97.4%, respectively, at 24 months. Motor function was normal in 64.3% from Rotterdam and in 81.6% from Rome at 12 months and in 45.7 and 89.8%, respectively, at 24 months. Longer length of hospital stay (LoS) was associated with worse cognitive outcome and motor function; LoS, low socioeconomic status, and ethnicity were associated with lower cognition. Conclusions: At 2 years, most CDH patients have normal cognition, but are at risk for motor function delay. Due to differences in outcomes between centers, careful interpretation is needed before conclusions can be drawn for other centers. Future multicenter collaboration should not only focus on standardization of postnatal care, but also on international standardization of follow-up to identify risk factors and thereby reduce morbidity.

Congenital diaphragmatic hernia (CDH) presents with a broad spectrum of severity, depending on the degree of pulmonary hypoplasia and persistent pulmonary hypertension (PPH). It is currently not clear whether pulmonary hypertension may affect late morbidity. Aim of the present study was to evaluate the influence of PPH on mid-term morbidity in high-risk CDH survivors.All high-risk (prenatal diagnosis and/or respiratory symptoms within 6 h of life) CDH survivors, treated between 2004 and 2008 in our Department were followed up in a multidisciplinary outpatient clinic as part of a longitudinal prospective study. Auxological, gastroesophageal, pulmonary and orthopedic evaluations were done at specific time-points (at 6, 12, and 24 months of age). Patients were grouped depending on the presence/absence of pulmonary hypertension (defined by expert pediatric cardiologists after echocardiography). Paired t-test and Fisher's exact test were used as appropriate. P0.05 was considered significant.70 survivors out of a total of 95 high-risk CDH infants treated in our Department attended our follow-up clinic and were prospectively evaluated. 17 patients were excluded from the present study because no clear data was available regarding the presence/absence of PPH during the perinatal period. Moreover, 9 infants were not enrolled because they did not reach at least 6 months of age. A total of 44 survivors were finally enrolled since they met the inclusion criteria. 26 infants did not present with PPH during the first hospital admission, while 18 had PPH. The 2 groups did not differ with regard to any of the outcomes considered at follow-up (p0.2).In our cohort of high-risk CDH survivors, PPH was not found to affect late sequelae at mid-term follow-up. This may indicate that postnatal pulmonary development is not (always) influenced by perinatal PPH. Nevertheless, a longer follow-up and more patients are needed to properly quantify possible late problems in high-risk CDH survivors with associated neonatal PPH.

Patients operated on for congenital pulmonary malformations (CPM) have excellent survival rates, but little is known about long-term morbidity. Our aim is to report the sequelae in patients operated on for CPM in infancy and to define factors that may influence their outcome.All patients operated on for major congenital anomalies are followed in a dedicated outpatient program and evaluated at 6, 24, and 48 months of life (corrected for gestational age) and at school age at 4, 6, 8, and 12 years of life. The data are prospectively collected. Patients operated on for CPM and enrolled in the follow-up clinic between January 2004 and December 2010 are compared with a control group of term infants operated on for inguinal hernia, without other major congenital or acquired abnormalities. The two groups were compared for auxological, respiratory, and orthopedic outcome.In the study period, 76 consecutive patients with CPM attended our dedicated follow-up clinic. Eight non-operated patients were excluded from the study. Age at follow-up was 82.0 (56.1-103.7) months in CPM patients and 83.5 (75.2-90.4) months in controls (P = 0.79). Fifty-three patients with CPM (78%) had one or more clinical or radiological abnormality versus six (16%) control patients (OR [95%CI] 16.5 [5.8-47.2]; P 0.0001).Over 50% of patients with CPM present long-term sequelae, regardless type of malformation. Therefore, long-term follow-up of patients operated on for CPM is recommended. Further studies are needed to define if, in asymptomatic patients, surgery may modify the natural history of CPM. Pediatr Pulmonol. 2016;51:1367-1372. © 2016 Wiley Periodicals, Inc.

Feeding and swallowing disorders are commonly seen in clinical practice in infants and children treated for esophageal atresia with or without tracheoesophageal fistula. Nevertheless, only few authors have addressed these issues. This review aims to describe the feeding and swallowing disorders encountered, focusing on pathophysiology, normal development of swallowing and feeding abilities, and possible rehabilitation therapies to prevent or correct these disorders.

Background Since mortality in congenital diaphragmatic hernia (CDH) is decreasing, morbidity such as neurodevelopmental delay receives increased attention. We evaluated neurodevelopmental outcome in high-risk CDH patients treated according to the Euroconsortium standardised neonatal treatment protocol (Reiss, 2010). Methods Observational, prospective cohort study in CDH patients treated in two level-III university hospitals (Rotterdam (ECMO available) and Rome (no ECMO available)) between January 2009–April 2011. Sixty-three patients underwent standardised neurodevelopmental assessment at corrected age of 12 and 24 months by using Bayley Scales of Infant Development (BSID)-II-NL (Rotterdam; Dutch reference data) or BSID-III (Rome; US reference data). Both centres were analysed separately due to differences in assessment instruments. Results are presented as n (%), mean (SD) or median (range). Results Mortality was 24.1% and 20.5% in Rotterdam and Rome, respectively. Baseline characteristics were for Rotterdam (n = 36)/Rome (n = 27): observed to expected lung-to-head ratio 53.8% (19.1)/55.4% (15.8), p = 0.67; SNAP-II score (19 (0–40)/7 (0–25), p Conclusions At two years, the majority of CDH patients have normal cognition, but they are at risk for motor function delay. Future multicenter collaboration should focus on continuation of standardisation of treatment and follow-up to identify risk factors and reduce morbidity.