Bambino Gesu Research Childrens Hospital

Rome, Italy

Bambino Gesu Research Childrens Hospital

Rome, Italy
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Bagolan P.,Bambino Gesu Research Childrens Hospital | Valfre L.,Bambino Gesu Research Childrens Hospital | Morini F.,Bambino Gesu Research Childrens Hospital | Conforti A.,Bambino Gesu Research Childrens Hospital
Diseases of the Esophagus | Year: 2013

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. © 2013 Wiley Periodicals, Inc. and the International Society for Diseases of the Esophagus.


Giuliani S.,St George's, University of London | Decker E.,St George's, University of London | Leva E.,Fondazione IRCCS Ca Granda | Riccipetitoni G.,Buzzi Childrens Hospital | Bagolan P.,Bambino Gesu Research Childrens Hospital
Journal of Pediatric Surgery | Year: 2016

Background: The aim of the study was to assess current international practice in the long term follow-up, in managing active problems and transition of care for teenagers born with anorectal malformations (ARM). Methods: An original survey was administered to delegates attending two large colorectal surgical meetings in 2015. The 21 questions covered long term follow-up, specific issues for teenagers and transition of care. Results: 96/236 delegates completed the survey. Follow-up was routinely suspended before 10 year of age by 33% of respondents. 90% of them did not use a scoring system to assess or risks stratify patients, despite 81% stating that an objective score would be beneficial. 40% of respondents felt that >. 30% of their teenagers had ongoing active medical or psychosocial issues. 42% thought their patients were not ready to be transitioned. The process of transition should start around 13-16 years according to 54% of respondents. 72% had no protocol for transition and 82% did not hold multidisciplinary meetings with adult practitioners before transition. Conclusions: International consensus on the following aspects of the care in ARM is needed: structured long term follow-up, objective assessment and risk stratification scores, pathways of transition and methods to prepare patients, parents and adult practitioners. © 2016 Elsevier Inc.


PubMed | St George's, University of London, Bambino Gesu Research Childrens Hospital, Buzzi Childrens Hospital and Fondazione IRCCS Ca Granda
Type: Journal Article | Journal: Journal of pediatric surgery | Year: 2016

The aim of the study was to assess current international practice in the long term follow-up, in managing active problems and transition of care for teenagers born with anorectal malformations (ARM).An original survey was administered to delegates attending two large colorectal surgical meetings in 2015. The 21 questions covered long term follow-up, specific issues for teenagers and transition of care.96/236 delegates completed the survey. Follow-up was routinely suspended before 10 year of age by 33% of respondents. 90% of them did not use a scoring system to assess or risks stratify patients, despite 81% stating that an objective score would be beneficial. 40% of respondents felt that >30% of their teenagers had ongoing active medical or psychosocial issues. 42% thought their patients were not ready to be transitioned. The process of transition should start around 13-16 years according to 54% of respondents. 72% had no protocol for transition and 82% did not hold multidisciplinary meetings with adult practitioners before transition.International consensus on the following aspects of the care in ARM is needed: structured long term follow-up, objective assessment and risk stratification scores, pathways of transition and methods to prepare patients, parents and adult practitioners.


PubMed | Bambino Gesu Research Childrens Hospital, Spedali Civili Hospital and University of Brescia
Type: Journal Article | Journal: Journal of pediatric surgery | Year: 2016

The management of newborns with esophageal atresia (EA) and right aortic arch (RAA) is still an unsolved problem. This study provides a systematic review of epidemiology, diagnosis, management and short-term results of children with EA and RAA.The PubMed database was searched for original studies on children with EA and RAA. In each study, data were extracted for the following outcomes: number of patients, associated anomalies, type of surgical repair, morbidity and mortality rate.Eight studies were selected, including 54 patients with EA and RAA. RAA was encountered in 3.6% of infants. Preoperative detection of RAA was reported in 7 of them. In these patients, primary anastomosis was achieved through the right approach in 3 (thoracotomy in 2 and thoracoscopy in 1) while the left approach was the primary choice in 4 (thoracotomy in 2 and thoracoscopy in 2). No significant differences were found between the right and left approaches with regard to leaks (P=0.89), strictures (P=1) or mortality (P=1). In 47/54 patients (87%) RAA was noted during right thoracotomy, and primary anastomosis was achieved through the same approach in 29 (61.7%); conversion to other approaches (left thoracotomy or esophageal substitution) was performed in 15 children (38.3%). No significant differences were found between primary left thoracotomy (LT) and LT after RT with regard to leaks (P=0.89), strictures (P=1) or mortality (P=1).Skills and preferences of the surgeon still guide the choice of surgical approach even when preoperatively faced with RAA. A multicenter, prospective randomized study is strongly required.


PubMed | Bambino Gesu Research Childrens Hospital
Type: Journal Article | Journal: Journal of pediatric surgery | Year: 2016

Newborn babies with esophageal atresia/tracheoesophageal fistula (EA/TEF) are prone to respiratory tract disorders. Functional residual capacity (FRC) and lung clearance index (LCI) are commonly considered useful and sensitive tools to investigate lung function and early detecting airways diseases. The aim of the present study is to report the first series of EA/TEF infants prospectively evaluated for FRC and LCI.Prospective observational cohort study of all patients treated for EA/TEF. Lung volume and ventilation inhomogeneity were measured by helium gas dilution technique using an ultrasonic flow meter. Babies were studied both in assisted controlled ventilation (sedated) and in spontaneous breathing (quiet sleep). Three consecutive FRC and LCI measurements were collected for each test at three different time points: before surgery (T0), 24hours after surgery (T1) and after extubation (T2).16 EA newborns were eligible for the study between December 2011 and July 2013. Three were excluded because of technical problems. At T0 FRC values were in the normal range regardless the presence of TEF but worsened afterwards at T1, with a subsequent recovering after extubation; a significant improvement after surgery was observed concerning LCI while no differences were found in tidal volume.Helium gas dilution technique is a suitable method to measure the effect of surgery on lung physiology, even in ventilated infants with EA. The changes observed could be related to the ventilatory management and lung compression during surgical procedure.

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