In patients a ected by CDH, survival beyond the neonatal period continues to increase thanks to t... more In patients a ected by CDH, survival beyond the neonatal period continues to increase thanks to technological and pharmacological improvements. Conversely, patients, families and caregivers are more and more frequently facing "new" complex late comorbidities, including chronic pulmonary and cardiac dysfunctions, neurodevelopmental challenges, and specific nutritional requirements, that often require ongoing long-term medical or surgical care. Therefore, late morbidity is now a key focus in clinical care of CDH. The aims of this paper are to stress some of the most important "unsolved problems" for CDH patients at long-term follow-up.
European Journal of Pediatric Surgery, Jul 17, 2023
Introduction The long-term outcomes of children who underwent surgery for congenital cystic adeno... more Introduction The long-term outcomes of children who underwent surgery for congenital cystic adenomatoid malformation of the lung (CCAML) are not well documented, particularly regarding orthopaedic and respiratory follow-up (FU). The aim of this study was to assess the long-term pulmonary and orthopaedic outcomes of surgically treated CCAML in newborns, infants, and toddlers. Materials and Methods Retrospective examination of prospectively recorded data of consecutive patients with CCAML who underwent surgery at our tertiary referral institution from January 2000 to December 2015 (newborns, infants, and toddlers). Clinical, radiological, and surgical data, as well as FU data were revised. A multidisciplinary team followed the patients after discharge at scheduled time points. Results Seventy-seven patients were included. After surgery, patients were followed for a median of 8 years (range: 1–19 years) until they reached a median age of 8 years (range: 2–19 years). Thirty patients (39%) developed wheezing and 21 (27%) had lower respiratory tract infections (LRTIs) within 4 years of age. However, more than 50% of patients with respiratory symptoms underwent complete remission in the following 4 years. Thirty-one patients (40%) developed at least one minimal musculoskeletal deformity. Eighteen (23%) had scoliosis, 17 (22%) thoracic asymmetry, 10 (12%) pectus excavatum, and 5 (6%) winged scapula. Conclusions Patients operated for CCAML had good overall outcomes despite pulmonary symptoms and musculoskeletal sequelae. Even though these issues are frequently paucisymptomatic, trying to use less-invasive procedures (such as minimally axillary open “muscle-sparing” thoracotomy or thoracoscopy) may reduce this burden. A structured multidisciplinary FU is required.
ObjectiveTo evaluate fetal brain development using MRI (magnetic resonance imaging) in CDH (conge... more ObjectiveTo evaluate fetal brain development using MRI (magnetic resonance imaging) in CDH (congenital diaphragmatic hernia).Methods52 isolated left CDH and 104 control fetuses were imaged using MRI. Brain morphometry (Biparietal diameter—BPD, brain fronto‐occipital diameter—BFOD, third ventricle, posterior ventricles, transcerebellar diameter—TCD, anteroposterior and craniocaudal cerebellar vermis diameter—AP and CC) and cortical structures (bilateral cingulate fissure—CF, insular fissure—IF, insular depth ‐ ID) were compared with controls using Mann–Whitney test.ResultsMedian gestational age at MRI (p = 0.95)and the median biparietal diameter (p = 0.737) were comparable. Among morphometric parameters, only the brain fronto‐occipital diameter was significantly smaller in CDH (p = 0.001) and the third ventricle was significantly greater in CDH (<0.0001). Among cortical structures, the cingulate and insular fissures were significantly deeper in CDH fetuses (p < 0.0001) as the i...
The best treatment of non-palpable testes is currently argument of debate. The aim of present stu... more The best treatment of non-palpable testes is currently argument of debate. The aim of present study was to describe authors' experience in surgical treatment with inguinal standard orchidopexy of non-palpable testes. In the last 17 years we have treated 2002 cryptorchid testes, among these 327 (16.33%) were non palpable. Age and distribution of cryptorchid testes was: 0-1 y (165 NPT), 1-2 y (84 NPT), 2-5 y (43 NPT), 5-10 y (16 NPT) and >10 y (19 NPT). Non-palpable testes were diagnosed and treated earlier (76.14% in the first two years). At surgical examination 204 (62.38%) were intrabdominal, 80 (24.46%) were atrophic and 43 (13.14%) vanishing. Among atrophic testes 54 (67.5%) were intracanicular, 21 (26.5%) were at the external inguinal ring, 4 (5%) were intrabdominal and 1 (1.25%) ectopic; among vanishing testes 22 (51.16%) were intrabdominal, 14 (32.55%) intracanicular and 7 (16.27%) at the external ring of inguinal canal. Atrophic and vanishing testes were in intrabdomin...
Feeding and swallowing disorders are commonly seen in clinical practice in infants and children t... more 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 Oesophageal atresia (OA) is characterized by a significant morbidity during the neonat... more 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 potentia...
Background: A high prevalence (9.5-30%) of eosinophilic esophagitis (EoE) in patients with esopha... more 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 < 0.0001). PPIresponder patients were significantly more prevalent in EA/EoE group (p ¼ 0.045). Conclusion: Our data confirm that EA patients are at high risk for developing EoE. High incidence, early onset, and high prevalence of PPI-responders might suggest that esophageal motility disorders interact to increase propensity to EoE in EA patients. However, our study also suggests that overdiagnosis of EoE may occur in EA and that adapted criteria for EoE diagnosis should be developed for EA patients.
Background Esophageal atresia (EA) prognosis have improved significantly over the past three deca... more 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 bet...
Introduction Since recently, after detection of eosinophilic predominant inflammation of esophagu... more 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 charact...
The Journal of Maternal-Fetal & Neonatal Medicine, 2018
Objectives:To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by th... more Objectives:To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by the introduction of a new ultrasonographic (US) marker, defined as mediastinal shift angle (MSA) and to evaluate its ability in predicting postnatal survival at discharge. Methods:Twentyfour consecutive fetuses from singleton pregnancies with isolated left-sided CDH were included in the study group and then subdivided in group A (n. 16 survivors) and group B (n.8 non survivors).
Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal re... more 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 Ն3 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.
Background: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) ... more Background: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care. Methods: A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015. Results: 69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%). Conclusions: Our study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.
In patients a ected by CDH, survival beyond the neonatal period continues to increase thanks to t... more In patients a ected by CDH, survival beyond the neonatal period continues to increase thanks to technological and pharmacological improvements. Conversely, patients, families and caregivers are more and more frequently facing "new" complex late comorbidities, including chronic pulmonary and cardiac dysfunctions, neurodevelopmental challenges, and specific nutritional requirements, that often require ongoing long-term medical or surgical care. Therefore, late morbidity is now a key focus in clinical care of CDH. The aims of this paper are to stress some of the most important "unsolved problems" for CDH patients at long-term follow-up.
European Journal of Pediatric Surgery, Jul 17, 2023
Introduction The long-term outcomes of children who underwent surgery for congenital cystic adeno... more Introduction The long-term outcomes of children who underwent surgery for congenital cystic adenomatoid malformation of the lung (CCAML) are not well documented, particularly regarding orthopaedic and respiratory follow-up (FU). The aim of this study was to assess the long-term pulmonary and orthopaedic outcomes of surgically treated CCAML in newborns, infants, and toddlers. Materials and Methods Retrospective examination of prospectively recorded data of consecutive patients with CCAML who underwent surgery at our tertiary referral institution from January 2000 to December 2015 (newborns, infants, and toddlers). Clinical, radiological, and surgical data, as well as FU data were revised. A multidisciplinary team followed the patients after discharge at scheduled time points. Results Seventy-seven patients were included. After surgery, patients were followed for a median of 8 years (range: 1–19 years) until they reached a median age of 8 years (range: 2–19 years). Thirty patients (39%) developed wheezing and 21 (27%) had lower respiratory tract infections (LRTIs) within 4 years of age. However, more than 50% of patients with respiratory symptoms underwent complete remission in the following 4 years. Thirty-one patients (40%) developed at least one minimal musculoskeletal deformity. Eighteen (23%) had scoliosis, 17 (22%) thoracic asymmetry, 10 (12%) pectus excavatum, and 5 (6%) winged scapula. Conclusions Patients operated for CCAML had good overall outcomes despite pulmonary symptoms and musculoskeletal sequelae. Even though these issues are frequently paucisymptomatic, trying to use less-invasive procedures (such as minimally axillary open “muscle-sparing” thoracotomy or thoracoscopy) may reduce this burden. A structured multidisciplinary FU is required.
ObjectiveTo evaluate fetal brain development using MRI (magnetic resonance imaging) in CDH (conge... more ObjectiveTo evaluate fetal brain development using MRI (magnetic resonance imaging) in CDH (congenital diaphragmatic hernia).Methods52 isolated left CDH and 104 control fetuses were imaged using MRI. Brain morphometry (Biparietal diameter—BPD, brain fronto‐occipital diameter—BFOD, third ventricle, posterior ventricles, transcerebellar diameter—TCD, anteroposterior and craniocaudal cerebellar vermis diameter—AP and CC) and cortical structures (bilateral cingulate fissure—CF, insular fissure—IF, insular depth ‐ ID) were compared with controls using Mann–Whitney test.ResultsMedian gestational age at MRI (p = 0.95)and the median biparietal diameter (p = 0.737) were comparable. Among morphometric parameters, only the brain fronto‐occipital diameter was significantly smaller in CDH (p = 0.001) and the third ventricle was significantly greater in CDH (<0.0001). Among cortical structures, the cingulate and insular fissures were significantly deeper in CDH fetuses (p < 0.0001) as the i...
The best treatment of non-palpable testes is currently argument of debate. The aim of present stu... more The best treatment of non-palpable testes is currently argument of debate. The aim of present study was to describe authors' experience in surgical treatment with inguinal standard orchidopexy of non-palpable testes. In the last 17 years we have treated 2002 cryptorchid testes, among these 327 (16.33%) were non palpable. Age and distribution of cryptorchid testes was: 0-1 y (165 NPT), 1-2 y (84 NPT), 2-5 y (43 NPT), 5-10 y (16 NPT) and >10 y (19 NPT). Non-palpable testes were diagnosed and treated earlier (76.14% in the first two years). At surgical examination 204 (62.38%) were intrabdominal, 80 (24.46%) were atrophic and 43 (13.14%) vanishing. Among atrophic testes 54 (67.5%) were intracanicular, 21 (26.5%) were at the external inguinal ring, 4 (5%) were intrabdominal and 1 (1.25%) ectopic; among vanishing testes 22 (51.16%) were intrabdominal, 14 (32.55%) intracanicular and 7 (16.27%) at the external ring of inguinal canal. Atrophic and vanishing testes were in intrabdomin...
Feeding and swallowing disorders are commonly seen in clinical practice in infants and children t... more 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 Oesophageal atresia (OA) is characterized by a significant morbidity during the neonat... more 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 potentia...
Background: A high prevalence (9.5-30%) of eosinophilic esophagitis (EoE) in patients with esopha... more 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 < 0.0001). PPIresponder patients were significantly more prevalent in EA/EoE group (p ¼ 0.045). Conclusion: Our data confirm that EA patients are at high risk for developing EoE. High incidence, early onset, and high prevalence of PPI-responders might suggest that esophageal motility disorders interact to increase propensity to EoE in EA patients. However, our study also suggests that overdiagnosis of EoE may occur in EA and that adapted criteria for EoE diagnosis should be developed for EA patients.
Background Esophageal atresia (EA) prognosis have improved significantly over the past three deca... more 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 bet...
Introduction Since recently, after detection of eosinophilic predominant inflammation of esophagu... more 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 charact...
The Journal of Maternal-Fetal & Neonatal Medicine, 2018
Objectives:To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by th... more Objectives:To quantify mediastinal shift in isolated congenital diaphragmatic hernia (CDH), by the introduction of a new ultrasonographic (US) marker, defined as mediastinal shift angle (MSA) and to evaluate its ability in predicting postnatal survival at discharge. Methods:Twentyfour consecutive fetuses from singleton pregnancies with isolated left-sided CDH were included in the study group and then subdivided in group A (n. 16 survivors) and group B (n.8 non survivors).
Long-gap esophageal atresia (LGEA) is still a major surgical challenge. Options for esophageal re... more 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 Ն3 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.
Background: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) ... more Background: Children with congenital esophageal atresia (EA) and tracheoesophageal fistula (TEF) have chronic respiratory symptoms including recurrent pneumonia, wheezing and persistent cough. The aim of this study is to describe the clinical findings of a large group of children with EA and TEF surgically corrected and the instrumental investigation to which they have undergone in order to better understand the patient's needs and harmonize the care. Methods: A retrospective data collection was performed on 105 children with EA and TEF followed at Department of Pediatric Medicine of Bambino Gesù Children's Hospital (Rome, Italy) between 2010 and 2015. Results: 69/105 (66%) children reported lower respiratory symptoms with a mean age onset of 2.2 ± 2.5 years and only 63/69 (91%) performed specialist assessment at Respiratory Unit. Recurrent pneumonia (33%) and wheezing (31%) were the most reported symptoms. The first respiratory evaluation was performed after surgically correction of gastroesophageal reflux (GER) at mean age of 3.9 ± 4.2 years. Twenty nine patients have undergone to chest CT with contrast enhancement detecting localized atelectasis (41%), residual tracheal diverticulum (34%), bronchiectasis (31%), tracheal vascular compression (21%), tracheomalacia (17%) and esophageal diverticulum (14%). Fifty three patients have undergone to airways endoscopy detecting tracheomalacia (66%), residual tracheal diverticulum (26%), recurrent tracheoesophageal fistula (19%) and vocal cord paralysis (11%). Conclusions: Our study confirms that respiratory symptoms often complicate EA and TEF; their persistence despite medical and surgical treatment of GER means that other etiological hypothesis must be examined and that a complete respiratory diagnostic work up must be considered.
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Papers by Laura Valfrè