Publications by authors named "Francesco Morini"

80 Publications

Growth and morbidity in infants with Congenital Diaphragmatic Hernia according to initial lung volume: A pilot study.

J Pediatr Surg 2021 Jul 8. Epub 2021 Jul 8.

Neonatal Surgery Unit, Medical and Surgical Department of Fetus - Newborn - Infant, "Bambino Gesù" Children's Hospital  IRCCS, Rome, Italy.

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.
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http://dx.doi.org/10.1016/j.jpedsurg.2021.06.013DOI Listing
July 2021

Predictive value of spinal bone anomalies for spinal cord abnormalities in patients with anorectal malformations.

J Pediatr Surg 2021 May 24. Epub 2021 May 24.

Medical and Surgical Department of the Fetus-Newborn-Infant, Newborn Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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 X 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.
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http://dx.doi.org/10.1016/j.jpedsurg.2021.05.011DOI Listing
May 2021

Randomized Trial of Fetal Surgery for Moderate Left Diaphragmatic Hernia.

N Engl J Med 2021 07 8;385(2):119-129. Epub 2021 Jun 8.

From the Department of Obstetrics and Gynecology, University Hospitals KU Leuven (J.A.D., P.L.J.D.) and Academic Department of Development and Regeneration, Biomedical Sciences, KU Leuven, Leuven, Belgium (J.A.D., B.V.C., P.L.J.D.); Hospital Antoine-Béclère, Université Paris-Saclay, Clamart (A.B.), and Necker-Enfants Malades Hospital, Paris (Y.V.) - both in France; Hospital Clinic and Sant Joan de Déu, Barcelona (E.G.); Institute for Women's Health, University College London Hospital (J.A.D.) and King's College Hospital(K.H.N.) - both in London; the University Hospital Bonn, Bonn, Germany (C.B.); Hospital Maggiore Policlinico, Milan (N.P.), and Bambino Gesù Children's Hospital, Rome (F.M.) - both in Italy; Baylor College of Medicine and Texas Children's Hospital (M.B.) and Children's Memorial Hermann Hospital (A.J.) - all in Houston; Mater Mothers' Hospital, Brisbane, QLD, Australia (G.J.G.); the Medical University of Warsaw, Warsaw, Poland (M.W.); and Erasmus MC-University Medical Center Rotterdam, Rotterdam, the Netherlands (P.L.J.D.).

Background: Fetoscopic endoluminal tracheal occlusion (FETO) has been associated with increased postnatal survival among infants with severe pulmonary hypoplasia due to isolated congenital diaphragmatic hernia on the left side, but data are lacking to inform its effects in infants with moderate disease.

Methods: In this open-label trial conducted at many centers with experience in FETO and other types of prenatal surgery, we randomly assigned, in a 1:1 ratio, women carrying singleton fetuses with a moderate isolated congenital diaphragmatic hernia on the left side to FETO at 30 to 32 weeks of gestation or expectant care. Both treatments were followed by standardized postnatal care. The primary outcomes were infant survival to discharge from a neonatal intensive care unit (NICU) and survival without oxygen supplementation at 6 months of age.

Results: In an intention-to-treat analysis involving 196 women, 62 of 98 infants in the FETO group (63%) and 49 of 98 infants in the expectant care group (50%) survived to discharge (relative risk , 1.27; 95% confidence interval [CI], 0.99 to 1.63; two-sided P = 0.06). At 6 months of age, 53 of 98 infants (54%) in the FETO group and 43 of 98 infants (44%) in the expectant care group were alive without oxygen supplementation (relative risk, 1.23; 95% CI, 0.93 to 1.65). The incidence of preterm, prelabor rupture of membranes was higher among women in the FETO group than among those in the expectant care group (44% vs. 12%; relative risk, 3.79; 95% CI, 2.13 to 6.91), as was the incidence of preterm birth (64% vs. 22%, respectively; relative risk, 2.86; 95% CI, 1.94 to 4.34), but FETO was not associated with any other serious maternal complications. There were two spontaneous fetal deaths (one in each group) without obvious cause and one neonatal death that was associated with balloon removal.

Conclusions: This trial involving fetuses with moderate congenital diaphragmatic hernia on the left side did not show a significant benefit of FETO performed at 30 to 32 weeks of gestation over expectant care with respect to survival to discharge or the need for oxygen supplementation at 6 months. FETO increased the risks of preterm, prelabor rupture of membranes and preterm birth. (Funded by the European Commission and others; TOTAL ClinicalTrials.gov number, NCT00763737.).
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http://dx.doi.org/10.1056/NEJMoa2026983DOI Listing
July 2021

Morphometric Analysis of Brain in Newborn with Congenital Diaphragmatic Hernia.

Brain Sci 2021 Apr 2;11(4). Epub 2021 Apr 2.

Medical Physics Department, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Congenital diaphragmatic hernia (CDH) is a severe pediatric disorder with herniation of abdominal viscera into the thoracic cavity. Since neurodevelopmental impairment constitutes a common outcome, we performed morphometric magnetic resonance imaging (MRI) analysis on CDH infants to investigate cortical parameters such as cortical thickness (CT) and local gyrification index (LGI). By assessing CT and LGI distributions and their correlations with variables which might have an impact on oxygen delivery (total lung volume, TLV), we aimed to detect how altered perfusion affects cortical development in CDH. A group of CDH patients received both prenatal (i.e., fetal stage) and postnatal MRI. From postnatal high-resolution T2-weighted images, mean CT and LGI distributions of 16 CDH were computed and statistically compared to those of 13 controls. Moreover, TLV measures obtained from fetal MRI were further correlated to LGI. Compared to controls, CDH infants exhibited areas of hypogiria within bilateral fronto-temporo-parietal labels, while no differences were found for CT. LGI significantly correlated with TLV within bilateral temporal lobes and left frontal lobe, involving language- and auditory-related brain areas. Although the causes of neurodevelopmental impairment in CDH are still unclear, our results may suggest their link with altered cortical maturation and possible impaired oxygen perfusion.
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http://dx.doi.org/10.3390/brainsci11040455DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8065764PMC
April 2021

You are never too small to make the difference A message for everyone and a push for pediatric surgeons in training.

Semin Pediatr Surg 2021 Feb 30;30(1):151016. Epub 2021 Jan 30.

Medical and Surgical Deparment of the Fetus, Newborn and Infant, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy.

Surgery, and training in surgery, is complex as it requires to master and to transmit both technical and non-technical skills. This is particularly true when the patient is a child with its family. Academies are called to ensure that the skills needed to guarantee the optimal care to the ill child, and to its parents, are conveyed to the new generations. In this paper, after shortly reviewing the history of academic pediatric surgery and the structure of pediatric surgery training in Italy, we will give a personal view on some of the most important non-technical challenges a young pediatric surgeon will face during his/her career, that make this discipline one of the hardest and the most rewarding at the same time.
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http://dx.doi.org/10.1016/j.sempedsurg.2021.151016DOI Listing
February 2021

Surgical Management of Pediatric Inguinal Hernia: A Systematic Review and Guideline from the European Pediatric Surgeons' Association Evidence and Guideline Committee.

Eur J Pediatr Surg 2021 Feb 10. Epub 2021 Feb 10.

Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.

Introduction:  Inguinal hernia repair represents the most common operation in childhood; however, consensus about the optimal management is lacking. Hence, recommendations for clinical practice are needed. This study assesses the available evidence and compiles recommendations on pediatric inguinal hernia.

Materials And Methods:  The European Pediatric Surgeons' Association Evidence and Guideline Committee addressed six questions on pediatric inguinal hernia repair with the following topics: (1) open versus laparoscopic repair, (2) extraperitoneal versus transperitoneal repair, (3) contralateral exploration, (4) surgical timing, (5) anesthesia technique in preterm infants, and (6) operation urgency in girls with irreducible ovarian hernia. Systematic literature searches were performed using PubMed, MEDLINE, Embase (Ovid), and The Cochrane Library. Reviews and meta-analyses were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) statement.

Results:  Seventy-two out of 5,173 articles were included, 27 in the meta-analyses. Laparoscopic repair shortens bilateral operation time compared with open repair. In preterm infants, hernia repair after neonatal intensive care unit (NICU)/hospital discharge is associated with less respiratory difficulties and recurrences, regional anesthesia is associated with a decrease of postoperative apnea and pain. The review regarding operation urgency for irreducible ovarian hernia gained insufficient evidence of low quality.

Conclusion:  Laparoscopic repair may be beneficial for children with bilateral hernia and preterm infants may benefit using regional anesthesia and postponing surgery. However, no definite superiority was found and available evidence was of moderate-to-low quality. Evidence for other topics was less conclusive. For the optimal management of inguinal hernia repair, a tailored approach is recommended taking into account the local facilities, resources, and expertise of the medical team involved.
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http://dx.doi.org/10.1055/s-0040-1721420DOI Listing
February 2021

European Pediatric Surgeon' Association Survey on the Management of Short-Bowel Syndrome.

Eur J Pediatr Surg 2021 Feb 16;31(1):8-13. Epub 2020 Nov 16.

Department of Pediatrics, Intestinal Failure Program, Assistance Publique des Hôpitaux de Marseille, Marseille, France.

Introduction:  The aim of this study was to assess the management of short-bowel syndrome (SBS) at the time of primary surgery, and the strategies used to facilitate enteral autonomy depending on the institutional expertise.

Materials And Methods:  An online questionnaire was sent in 2019 to members of The European Pediatric Surgeons' Association.

Results:  Among the 65 responding members (26 countries, 85% from university hospitals), 57% manage less than three new patients with SBS per year (group A), and 43% at least three patients (group B). The cut-off of three patients treated yearly used in our study was defined after statistical analysis of different cut-offs. A multidisciplinary intestinal rehabilitation program is significantly more frequent in group B than in group A (85 and 53%, respectively;  = 0.009). Considering the primary surgical management of multiple intestinal atresia and congenital ultra-short bowel with jejunal atresia, primary surgical strategies to optimize bowel length are more often used in group B than group A ( = 0.09 and  = 0.04, respectively). A minimum of one intestinal lengthening procedure every 2 to 3 years is significantly more frequent in group B than group A (95 and 45%, respectively;  = 0.0013). Among the strategies used to promote intestinal adaptation, group B (35%) uses significantly more often glucagon-like peptide 2 analogs than group A (10%) ( = 0.02).

Conclusion:  Based on our survey, a minimum number of SBS patients treated yearly is required to manage this challenging disease according to up-to-date medical and surgical strategies. However, whatever their level of expertise is in managing SBS, most of pediatric surgeons are involved in the primary surgery. Medical education programs about SBS should be more largely available to pediatric surgeons.
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http://dx.doi.org/10.1055/s-0040-1721040DOI Listing
February 2021

Diagnostic Workup of Neonates With Esophageal Atresia: Results From the EUPSA Esophageal Atresia Registry.

Front Pediatr 2020 25;8:489. Epub 2020 Aug 25.

Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy.

Controversies exist on the optimal diagnostic workup for neonates with esophageal atresia (EA) with/without tracheoesophageal fistula (TEF). Aim of this study was to describe the current diagnostic policies in EA/TEF patients enrolled in an International multicenter registry. All patients consecutively registered from July 2014 to December 2017 in the EUPSA Esophageal Atresia Registry (EUPSA-EAR) were included in the study. Data related to diagnostic investigations among Centers forming the EUPSA-EAR were analyzed. During the study period, 374 consecutive patients were recorded by 23 Centers. The majority of patients underwent chest X-rays, echocardiography, abdominal ultrasound, and abdominal X-rays. Preoperative bronchoscopy and esophageal gap measurement were performed in one third of the patients. Present data from a large cohort of patients from the EUPSA-EAR show both inter-institutional and intra-institutional variability in diagnostic workup of patients with EA/TEF. Efforts should be made to develop guidelines on the diagnostic workup for EA/TEF patients.
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http://dx.doi.org/10.3389/fped.2020.00489DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7477332PMC
August 2020

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

Ital J Pediatr 2020 Sep 16;46(1):134. Epub 2020 Sep 16.

Department of Neonatal and Emergency Surgery, Meyer Children's Hospital, Florence, Italy.

Introduction: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy.

Methods: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units.

Results: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern
Conclusion: Bedside surgery is performed in the majority of the Italian pediatric surgery centers included in this census. The introduction of a national set of surgery guidelines would be widely welcomed.
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http://dx.doi.org/10.1186/s13052-020-00889-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7493058PMC
September 2020

Pediatric medical traumatic stress (PMTS) in parents of newborns with a congenital anomaly requiring surgery at birth.

J Pediatr Surg 2021 Mar 31;56(3):471-475. Epub 2020 Jul 31.

Unit of Clinical Psychology, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, Rome, Italy.

Background: 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.

Methods: 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).

Results: 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.

Conclusions: 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.

Level Of Evidence: Prognosis study, level II.
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http://dx.doi.org/10.1016/j.jpedsurg.2020.07.030DOI Listing
March 2021

Lack of Gut Secretory Immunoglobulin A in Memory B-Cell Dysfunction-Associated Disorders: A Possible Gut-Spleen Axis.

Front Immunol 2019 8;10:2937. Epub 2020 Jan 8.

Department of Molecular Medicine, Sapienza University, Rome, Italy.

B-1a B cells and gut secretory IgA (SIgA) are absent in asplenic mice. Human immunoglobulin M (IgM) memory B cells, which are functionally equivalent to mouse B-1a B cells, are reduced after splenectomy. To demonstrate whether IgM memory B cells are necessary for generating IgA-secreting plasma cells in the human gut. We studied intestinal SIgA in two disorders sharing the IgM memory B cell defect, namely asplenia, and common variable immune deficiency (CVID). Splenectomy was associated with reduced circulating IgM memory B cells and disappearance of intestinal IgA-secreting plasma cells. CVID patients with reduced circulating IgM memory B cells had a reduced frequency of gut IgA plasma cells and a disrupted film of SIgA on epithelial cells. Toll-like receptor 9 (TLR9) and transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI) induced IgM memory B cell differentiation into IgA plasma cells . In the human gut, TACI-expressing IgM memory B cells were localized under the epithelial cell layer where the TACI ligand a proliferation inducing ligand (APRIL) was extremely abundant. Circulating IgM memory B cell depletion was associated with a defect of intestinal IgA-secreting plasma cells in asplenia and CVID. The observation that IgM memory B cells have a distinctive role in mucosal protection suggests the existence of a functional gut-spleen axis.
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http://dx.doi.org/10.3389/fimmu.2019.02937DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6960143PMC
November 2020

Minimally Invasive Techniques for Hirschsprung Disease.

J Laparoendosc Adv Surg Tech A 2019 Dec 15;29(12):1605-1608. Epub 2019 Oct 15.

Neonatal Surgery Unit, Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Surgical treatment of Hirschsprung disease (HD) evolved in the last decades with the introduction of new innovative minimally invasive techniques. The aim of present study is to compare the results from two centers, applying similar minimal access approaches, total transanal endorectal pull-through (TEPT) and laparoscopic-assisted transanal pull-through (LA-TAPT). Data of all patients admitted between January 2011 and December 2016 in two Italian referral Hospitals for HD were retrospectively collected and analyzed. Exclusion criteria were as follows: redo procedure, patients with total colonic aganglionosis (ileostomy), patients lost at follow-up, or missing data. Patients who underwent TEPT and LA-TAPT were compared for gestational age, birth weight, age and weight at surgery, associated malformation, genetic syndrome, length of hospital stay, and early postoperative complications (within 30 days). Statistical analysis: Fisher's test and Mann-Whitney test; statistical significance set at  < .05. None of the patients who underwent TEPT required laparotomy or laparoscopic assistance. Patients who underwent TEPT had lower age ( = .001), lower body weight ( < .0001), a significant higher rate of associated congenital heart disease ( = .006), and longer postoperative stay. In addition, the prevalence of perianal dermatitis was higher in TEPT patients (17/61 [28%] versus 2/46 [4%],  = .001). The two groups were similar in terms of postoperative enterocolitis, redo surgery, anastomotic stenosis, and other infective complications. Both minimally invasive techniques allow surgical advantages and outcomes; TEPT avoids pneumoperitoneum and the transperitoneal approach, with no need for laparoscopic instrumentation. TEPT group had longer postoperative stay, probably due to the higher prevalence of associated cardiac anomalies, and higher prevalence of perianal dermatitis, probably due to the lower age at operation.
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http://dx.doi.org/10.1089/lap.2019.0165DOI Listing
December 2019

Intravenous Propofol Allows Fast Intubation in Neonates and Young Infants Undergoing Major Surgery.

Front Pediatr 2019 14;7:321. Epub 2019 Aug 14.

Department of Anesthesia and Critical Care, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy.

In selected surgical neonates and infants, the rapidity of induction and intubation may represent an important factor for their safety. Propofol is an anesthetic characterized by a rapid onset and fast recovery time that may reduce time of anesthetic induction and improve post-anesthetic outcome. The aim of this study was to evaluate the safety and efficacy of anesthesia induction in full-term neonates and young infants after propofol bolus administration. A retrospective case-control study including infants below 6 months of age, undergoing general anesthesia between 2011 and 2013, was carried out. Patients that received intravenous propofol bolus to induce anesthesia were compared to patients who received inhaled sevoflurane. Time to reach successful orotracheal intubation (OTI) was measured in seconds. The quality of OTI was defined as "excellent," "good," and "poor," based on established classification and was reported. Hemodynamic parameters as systolic blood pressure (SBP), diastolic blood pressure (DBP), pulse pressure (PP), heart rate (HR), and oxygen saturation (SaO2) were collected before OTI (t0), at OTI (t1), and at spontaneous breathing recovery (t2). Main adverse effects were recorded for both groups. Results are median (IQ range) or prevalence; < 0.05 was considered significant. 160 infants were enrolled in the study, 80 received propofol and 80 inhaled sevoflurane. Major surgery (involving organs in the thoracic, abdominal, or pelvic cavities) was performed in 64 and 54% of patients in the propofol and sevoflurane group, respectively ( = 0.07). Patients in the propofol group showed a shorter time for OTI [11.5 (4.0-65) vs. 360.0 (228.0-720.0) seconds, ( < 0.0001)]. No difference was found in the quality of OTI between the two groups. No significant complications were recorded in either group. Propofol is a safe and effective anesthetic in neonates and infants permitting rapid induction of anesthesia and rapid intubation, without negative impact on the quality of intubation and haemodynamic compromise.
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http://dx.doi.org/10.3389/fped.2019.00321DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6702265PMC
August 2019

Risk Factors for Surgical Site Infection in Neonates: A Systematic Review of the Literature and Meta-Analysis.

Front Pediatr 2019 29;7:101. Epub 2019 Mar 29.

Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Surgical site infections (SSI) contribute to postoperative morbidity and mortality in children. Our aim was to evaluate the prevalence and identify risk factors for SSI in neonates. Using a defined strategy, three investigators searched articles on neonatal SSI published since 2000. Studies on neonates and/or patients admitted to neonatal intensive care unit following cervical/thoracic/abdominal surgery were included. Risk factors were identified from comparative studies. Meta-analysis was conducted according to PRISMA guidelines using RevMan 5.3. Data are (mean ± SD) prevalence. Systematic review-of 885 abstracts screened, 48 studies (27,760 neonates) were included. The incidence of SSI was 5.6% (1,564 patients). SSI was more frequent in males (61.8%), premature babies (77.4%), and following gastrointestinal surgery (95.4%). Meta-analysis-10 comparative studies (16,442 neonates; 946 SSI 5.7%) showed that predictive factors for SSI development were gestational age, birth weight, age at surgery, length of surgical procedure, number of procedure per patient, length of preoperative hospital stay, and preoperative sepsis. Conversely, preoperative antibiotic use was not significantly associated with development of SSI. Younger neonates and those undergoing abdominal procedures are at higher risk for SSI. Given the lack of evidence-based literature, prospective studies may help determine the risk factors for SSI in neonates.
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http://dx.doi.org/10.3389/fped.2019.00101DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6449628PMC
March 2019

Management preferences in ECMO mode for congenital diaphragmatic hernia.

J Pediatr Surg 2019 May 31;54(5):903-908. Epub 2019 Jan 31.

University of California Irvine Medical Center, Department of Surgery, Orange, CA; Children's Hospital of Orange County, Division of Pediatric Surgery, Orange, CA.

Purpose: The purpose of this study was to identify management preferences that may exist in the care of infants with CDH receiving ECMO with emphasis on VV-ECMO.

Methods: A survey was created to measure treatment preferences regarding ECMO use in CDH. The survey was distributed to all APSA and ELSO/Euro-ELSO members via e-mail. Survey results were summarized using descriptive statistics.

Results: The survey had 230 respondents. The survey participants were surgeons (75%), neonatologists/intensivists (23%), and "other" (2%). The mean annual center volume was 11.6(±9.6) CDH cases, and the average number treated with ECMO was 4.5 (±6.4) cases/yr. The most agreed upon criteria for ECMO initiation were preductal O saturation <80% refractory to ventilator manipulation and medical therapy (89%), oxygenation index >40 (80%), severe air-leak (79%), and mixed acidosis (75%). Over 60% of respondents agreed the VV-ECMO would be optimum for average risk neonates. However, this preference diminished as the pre-ECMO level of cardiac support increased. When asked about why each respondent would choose VA-ECMO over VV-ECMO, the responses varied significantly between surgeons and non-surgeons.

Conclusion: While there seem to be areas of consensus among practitioners, such as criteria for initiation of ECMO, this survey revealed substantial variation in individual practice patterns regarding the use of ECMO for CDH.

Type Of Study: Qualitative, Survey.

Level Of Evidence: IV.
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http://dx.doi.org/10.1016/j.jpedsurg.2019.01.019DOI Listing
May 2019

Autologous Intestinal Reconstructive Surgery in the Management of Total Intestinal Aganglionosis.

J Pediatr Gastroenterol Nutr 2019 05;68(5):635-641

Section of Pediatric Surgery, Pediatric Liver and Gut Research Group, Children's Hospital, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.

Objectives: Total/near total intestinal aganglionosis (TIA/NTIA) is the most uncommon and life-threatening form of Hirschsprung disease (HD). The management of TIA/NTIA is challenging and the role of autologous intestinal reconstructive (AIR) surgery is controversial. The objective is to evaluate the effectiveness of AIR in patients with TIA/NTIA.

Methods: Records from children affected by TIA and enrolled in the multicenter international Pediatric Intestinal Rehabilitation and Transplantation Registry were retrospectively reviewed.

Results: Fourteen patients with TIA were identified. TIA diagnosis was confirmed histologically at the median age of 14 days of life. All received a proximal decompressive jejunostomy. Two patients died, 4 patients had satisfactory stoma output with enteral tolerance without additional procedures, 8 underwent 10 AIR procedures (4 Ziegler myotomy-myectomy, 3 transposition of aganglionic ileum with or without myotomy, 2 simple tapering, 1 longitudinal lengthening and tailoring procedure with associated myotomy). AIR significantly reduced median stoma output, from 197 to 31 mL · kg · day (P = 0.0001). The reduction was seen in all patients. In addition, AIR improved enteral tolerance in the long term in 5 of 8 patients (63%), and temporarily in 1, leading to a reduction of parenteral nutrition requirement from 100% to 70% (P = 0.0231).

Conclusions: AIR surgery in carefully selected patients may be useful and effective way to enhance residual bowel absorptive function and to reduce parenteral nutrition requirements. AIR and intestinal transplantation are complementary surgical tools in the complex treatment algorithm of TIA/NTIA.
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http://dx.doi.org/10.1097/MPG.0000000000002260DOI Listing
May 2019

Lung Transplantation for Late-Onset Pulmonary Hypertension in a Patient with Congenital Diaphragmatic Hernia.

European J Pediatr Surg Rep 2018 Jan 26;6(1):e100-e103. Epub 2018 Dec 26.

Department of Neonatal Surgery, Bambino Gesù Children's Hospital, Rome, Italy.

Lung hypoplasia and pulmonary hypertension (PH) in association with congenital diaphragmatic hernia (CDH) may cause fatal respiratory failure. Lung transplantation (Ltx) may represent an option for CDH-related end-stage pulmonary failure. The aim of this study is to report a patient with CDH who underwent Ltx or combined heart-lung transplantation (H-Ltx). Our patient was born at 33 weeks of gestation, with a prenatally diagnosed isolated left CDH. Twenty-four hours after birth, she underwent surgical repair of a type D defect (according to the CDH Study Group staging system). Postoperative course was unexpectedly uneventful, and she was discharged home at 58 days of life. Echocardiography before discharge was unremarkable. Periodic follow-up revealed gastroesophageal reflux (GER) and initial scoliosis. At the age of 10, she was readmitted for severe PH. Lung function progressively deteriorated, and at the age of 14, she underwent H-Ltx due to end-stage respiratory failure. After discharge, she developed recurrent respiratory tract infections, severe malnutrition, and drug-induced diabetes. Scoliosis and GER progressed, requiring posterior vertebral arthrodesis and antireflux surgery, respectively. Bronchiolitis obliterans further impaired her respiratory function, and though she had a second Ltx, she died at the age of 18, 4 and 1.5 years after the first and the second Ltx, respectively. Late-onset PH is an ominous complication of CDH. From our patient and the six further cases collected from the literature, Ltx may be considered as a last-resource treatment in CDH patients with irreversible and fatal respiratory failure, although its prognosis seems unfair.
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http://dx.doi.org/10.1055/s-0038-1675377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306278PMC
January 2018

Impact of the associated anorectal malformation on the outcome of spinal dysraphism after untethering surgery.

Pediatr Surg Int 2019 Feb 2;35(2):227-231. Epub 2018 Nov 2.

Department of Medical and Surgical Neonatology, Neonatal Surgery Unit, Bambino Gesù Children's Hospital, IRCCS, Piazza S. Onofrio, 4, 00165, Rome, Italy.

Purpose: To analyze the outcome after untethering surgery in patients with spinal dysraphism (SD), with or without associated anorectal malformation (ARM).

Methods: Patients operated on for SD, with (Group A) or without (Group B) associated ARM (1999-2015), were included. The post-operative outcome was analyzed in the two groups in terms of improving of clinical symptoms (neuro-motor deficits, bladder dysfunction, bowel dysfunction) and of instrumental examinations (urodynamics, bladder ultrasound, neurophysiology). Fisher's exact test and χ test were used as appropriate; p < 0.05 was considered statistically significant.

Main Results: Ten patients in Group A and 24 in Group B were consecutively treated. One patient was lost at follow up. Six patients (25%) in Group B underwent prophylactic surgery. The analysis of the pre-operative symptoms in the two groups showed that a significantly higher number of patients in group A needed bowel management and presented with neuro-motor deficits, compared to group B (p = 0.0035 and p = 0.04, respectively). Group A showed a significant post-operative neuro-motor improvement as compared to group B (p = 0.002).

Conclusions: Based on our results, untethering seems to be effective in neuro-motor symptoms in selected patients with ARM. In ARM patients, untethering surgery does not seem to benefit intestinal and urinary symptoms. The presence of the associated ARM does not seem to impact the medium-term outcome of patients operated for SD.
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http://dx.doi.org/10.1007/s00383-018-4400-8DOI Listing
February 2019

European Paediatric Surgeons' Association Survey on the Management of Pediatric Appendicitis.

Eur J Pediatr Surg 2019 Feb 15;29(1):53-61. Epub 2018 Aug 15.

Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada.

Aim:  To define patterns in the management of pediatric appendicitis.

Methods:  A total of 169 delegates from 42 (24 European) countries completed a validated survey administered at the EUPSA 2017 annual congress.

Results:  In the work-up of children with suspected acute appendicitis, most surgeons rely on full blood count (92%), C-reactive protein (82%), and abdominal ultrasonography (76%), but rarely on computed tomography scans or magnetic resonance imaging. In suspected simple appendicitis, most surgeons (76%) do not perform appendectomy at night in clinically stable patients and start antibiotic preoperatively (64%), but only 15% offer antibiotic therapy alone (no appendectomy). In suspected perforated appendicitis, 96% start antibiotic preoperatively, and 92% perform an appendectomy. Presence of phlegmon/abscess is the main contraindication to immediate surgery. In case of appendix mass, most responders (75%) favor a conservative approach and perform interval appendectomy always (56%) or in selected cases (38%) between 2 and 6 months from the first episode (81%). Children with large intraperitoneal abscesses are managed by percutaneous drainage (59% responders) and by surgery (37% responders). Laparoscopy is the preferred surgical approach for both simple (89%) and perforated appendicitis (81%). Most surgeons send the appendix for histology (96%) and pus for microbiology, if present (78%). At the end of the operation, 58% irrigate the abdominal cavity only if contaminated using saline solution (93%). In selected cases, 52% leave a drain in situ.

Conclusion:  Some aspects of appendicitis management lack consensus, particularly appendix mass and intraperitoneal abscess. Evidence-based guidelines should be developed, which may help standardize care and improve clinical outcomes.
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http://dx.doi.org/10.1055/s-0038-1668139DOI Listing
February 2019

Defining outcomes following congenital diaphragmatic hernia using standardised clinical assessment and management plan (SCAMP) methodology within the CDH EURO consortium.

Pediatr Res 2018 08 29;84(2):181-189. Epub 2018 May 29.

Department of Intensive Care and Pediatric Surgery, Erasmus MC-Sophia Children's Hospital University Medical Center Rotterdam, Rotterdam, The Netherlands.

Treatment modalities for neonates born with congenital diaphragmatic hernia (CDH) have greatly improved in recent times with a concomitant increase in survival. In 2008, CDH EURO consortium, a collaboration of a large volume of CDH centers in Western Europe, was established with a goal to standardize management and facilitate multicenter research. However, limited knowledge on long-term outcomes restricts the identification of optimal care pathways for CDH survivors in adolescence and adulthood. This review aimed to evaluate the current practice of long-term follow-up within the CDH EURO consortium centers, and to review the literature on long-term outcomes published from 2000 onward. Apart from having disease-specific morbidities, children with CDH are at risk for impaired neurodevelopmental problems and failure of educational attainments which may affect participation in society and the quality of life in later years. Thus, there is every reason to offer them long-term multidisciplinary follow-up programs. We discuss a proposed collaborative project using standardized clinical assessment and management plan (SCAMP) methodology to obtain uniform and standardized follow-up of CDH patients at an international level.
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http://dx.doi.org/10.1038/s41390-018-0063-3DOI Listing
August 2018

Pathogenetic and Prognostic Factors for Neonatal Gastric Perforation: Personal Experience and Systematic Review of the Literature.

Front Pediatr 2018 4;6:61. Epub 2018 Apr 4.

Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Introduction: Neonatal gastric perforation (NGP) is a rare entity. Our aim was to report our experience and review the recent literature to characterize NGP, describe associated factors, and define prognostic factors.

Materials And Methods: Retrospective review of all consecutive patients with NGP treated between June 2009 and December 2017 in a third level pediatric hospital. In addition, a systematic review of Medline and Scopus database was performed using a defined strategy. All articles referring to NGP published between 2005 and 2017 were retrieved. Variables considered: prematurity (<37 weeks gestation), birth weight (BW), Apgar score, prenatal complications, age at diagnosis, bag ventilation, pathogenetic events, site of perforation, treatment of perforation, sepsis, and outcome. Mann-Whitney or Fisher's test were used as appropriate. Results are median (range) or prevalence.

Results: Between 2009 and 2016 we treated 8 consecutive patients for NGP and 199 further cases were retrieved from the systematic review (total of 207 patients). Overall survival was 73%. Most frequently reported pathogenesis: iatrogenic (20 patients), hypoxic/ischemic or infection stress (13 patients), duodenal/jejunal obstruction (11 patients), drugs (11 patients), esophageal atresia (10 patients). 60% patients had only primary repair of the perforation as gastric surgery. Sepsis developed in 56 patients (34%).

Conclusion: Although the pathogenesis of NGP is pleomorphic, prematurity and low BWs are frequent in these patients. Reviewing our experience and the available literature, none of the variables considered, but sepsis was associated with mortality.
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http://dx.doi.org/10.3389/fped.2018.00061DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5893822PMC
April 2018

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

Am J Perinatol 2018 10 18;35(12):1168-1172. Epub 2018 Apr 18.

Unit of Clinical Psychology, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, Rome, Italy.

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-: 0.6842,  < 0.0001 and 12 months-: 0.4045,  = 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.
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http://dx.doi.org/10.1055/s-0038-1641591DOI Listing
October 2018

Surgery of Neonates: What's New in Dealing with Perioperative Complications?

Eur J Pediatr Surg 2018 04 22;28(2):131-132. Epub 2018 Mar 22.

Department of Medical and Surgical Neonatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy.

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http://dx.doi.org/10.1055/s-0038-1637767DOI Listing
April 2018

Perioperative Complications of Esophageal Atresia.

Eur J Pediatr Surg 2018 Apr 13;28(2):133-140. Epub 2018 Mar 13.

Neonatal Surgery Unit, Department of Medical and Surgical Neonatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy.

Current survival rate of infants with esophageal atresia with/without tracheoesophageal fistula (EA/TEF) exceeds 90% and great interest is dedicated to the long-term outcomes and complications. However, perioperative complications represent a significant burden to the EA/TEF infants and a technical challenge for the surgeon. In this review, we will focus on the most frequent perioperative complications specific of EA/TEF to describe their predisposing factors, possible preventive measures, and treatment options.
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http://dx.doi.org/10.1055/s-0038-1636941DOI Listing
April 2018

Long-term morbidity of congenital diaphragmatic hernia: A plea for standardization.

Semin Pediatr Surg 2017 Oct 20;26(5):301-310. Epub 2017 Sep 20.

Neonatal Surgery Unit, Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. Electronic address:

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.
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http://dx.doi.org/10.1053/j.sempedsurg.2017.09.002DOI Listing
October 2017

Treatment Strategies for Congenital Diaphragmatic Hernia: Change Sometimes Comes Bearing Gifts.

Front Pediatr 2017 14;5:195. Epub 2017 Sep 14.

Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Objective: To report treatment strategies' evolution and its impact on congenital diaphragmatic hernia (CDH) outcome.

Design: Registry-based cohort study using the CDH Study Group database, 1995-2013.

Setting: International multicenter database.

Patients: CDH patients entered into the registry. Late presenters or patients with very incomplete data were excluded. Patients were divided into three Eras (1995-2000; 2001-2006; 2007-2013).

Main Outcome Measures: Treatment strategies and outcomes. One-way ANOVA, X2 test, and X2 test for trend were used. A Sydak-adjusted  < 0.0027 was considered significant. Prevalence or mean (SE) are reported.

Results: Patients: 8,603; included: 7,716; Era I: 2,146; Era II: 2,572; Era III: 2,998. From Era I to Era III, significant changes happened. Some severity indicators such as gestational age, prevalence of prenatal diagnosis, and inborn patients significantly worsened. Also, treatment strategies such as the use of prenatal steroids and inhaled nitric oxide, age at operation, prevalence of minimal access surgery, and the use of surfactant significantly changed. Finally, length of hospital stay became significantly longer and survival to discharge slightly but significantly improved, from 67.7 to 71.4% ( for trend 0.0019).

Conclusion: Treatment strategies for patients registered since 1995 in the CDH Study Group significantly changed. Survival to discharge slightly but significantly improved.
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http://dx.doi.org/10.3389/fped.2017.00195DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5603669PMC
September 2017

Pediatric Intestinal Rehabilitation and Transplantation Registry: Initial Report from a European Collaborative Registry.

Eur J Pediatr Surg 2018 Feb 24;28(1):75-80. Epub 2017 Aug 24.

Department of Medical and Surgical Neonatology, Bambino Gesù Children's Research Hospital, Rome, Italy.

Introduction:  Short bowel syndrome (SBS) is the main cause of intestinal failure (IF) in the pediatric population. To promote the standardization of care of these patients, the registry of Pediatric Intestinal Rehabilitation and Transplantation (PIRAT) has been established. The aim of this study is to describe patients with IF using PIRAT database.

Materials And Methods:  Data from two tertiary care European referral Centers registered in PIRAT (https://www.studeon.eu/pirat) were analyzed (1994-2015). Neonatal SBS-related IF was defined as need for parenteral nutrition (PN) to sustain life and growth for more than 75 days, after extensive bowel resection during neonatal period. Data included patient demographics, disease at birth, residual small intestine, and intestinal autonomy (PN on/off).

Results:  In this study, 114 children with SBS-related IF were identified (male 60%). Median gestational age was 35.3 weeks (interquartile range [IQR]: 33.0-38.0); median birth weight was 2,440 g (IQR: 1,700-2,990). The main causes of SBS were intestinal atresia in 31 (27%), midgut volvulus in 29 (25%), necrotizing enterocolitis in 23 (20%), and gastroschisis in 12 (11%). Nine (7.9%) patients died on PN (six sepsis, two IF-associated liver disease, and one multiorgan failure). Median residual small bowel length was 46 cm (IQR: 13.0-92.5). Ileocecal valve was resected in 48 patients (42%). Intestinal autonomy was achieved in 68% patients.

Conclusion:  We present the web-based registry PIRAT and the first results of patients with IF registered from two European Centers. PIRAT could give the opportunity to create a dedicated international network (IF-net) to standardize, improve, and spread the therapeutic paths for the rare and heterogeneous condition of SBS-related IF.
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http://dx.doi.org/10.1055/s-0037-1605349DOI Listing
February 2018

Current Management of Congenital Pulmonary Airway Malformations: A "European Pediatric Surgeons' Association" Survey.

Eur J Pediatr Surg 2018 Feb 14;28(1):1-5. Epub 2017 Jul 14.

Department of Medical and Surgical Neonatology, Bambino Gesu Children's Research Hospital, Rome, Italy.

Aim:  To define current management of congenital pulmonary airway malformation (CPAM).

Methods:  A total of 181 European Pediatric Surgeons' Association members (91% senior) from 48 countries completed an online questionnaire.

Main Results:  Prenatal: 93% respondents work in centers with prenatal diagnosis facilities, and 27% in centers offering in utero surgery. Prenatal counseling is performed by 86% respondents, 22% of whom see >10 cases per year. Risk of single pre-/postnatal complications is deemed low (<5%) by more than 60% of respondents. Eighty-six percent respondents do not offer pregnancy termination for prenatally diagnosed CPAM. Fetal hydrops is the most frequent indication for termination (87%), followed by parental willingness (52%). Prenatal surgery is an option for 44% respondents, preferring thoracoamniotic shunt (82%).Postnatal: 75% respondents operate on asymptomatic patients, 18% before 6 months of age, 62% between 6 and 12 months of age, and 20% after 12 months of age. Risk of infection (86%), cancer (63%), and symptoms development (62%) are indications for surgery in asymptomatic CPAM. Sixty-three percent prefer a thoracotomy. Lobectomy is the preferred procedure (58% respondents). Motivations against surgery include lesion <1 cm (64%), risk of postoperative complications (37%), and lack of evidence favoring surgery (27%). Seventeen percent respondents have seen at least one patient with CPAM with lung cancer, in 89% of the cases within the CPAM. Of all the respondents, 83% and 22% offered dedicated follow-up and genetic screening, respectively.

Conclusion:  Current pre- and postnatal management of CPAM lacks uniformity, particularly for surgical indication, timing, and approach. Efforts should be made toward standardization. Risk of CPAM-associated cancer is not clear.
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http://dx.doi.org/10.1055/s-0037-1604020DOI Listing
February 2018

Ventilation modalities in infants with congenital diaphragmatic hernia.

Semin Pediatr Surg 2017 Jun 25;26(3):159-165. Epub 2017 Apr 25.

Division of Neonatology, Department of Pediatrics, Erasmus MC-Sophia Children's Hospital, University Medical Center, Rotterdam, The Netherlands.

Neonates with congenital diaphragmatic hernia are among the more complex patients to support with mechanical ventilation. They have particular features that add to the difficulties already present in the neonatal patient. A ventilation strategy tailored to the patient's underlying physiology rather than mode of ventilation is a crucial issue for clinicians treating these delicate patients.
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http://dx.doi.org/10.1053/j.sempedsurg.2017.04.003DOI Listing
June 2017
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