Guía de Práctica Clínica en atresia esofágica / Clinical practice guide on esophageal atresia

La atresia esofágica es una de las anomalías congénitas más frecuentes en la práctica quirúrgica neonatal. Se estima que tiene una incidencia de 1 por cada 3500 recién nacidos vivos a nivel mundial. La preparación de guías de actuación y protocolos asistenciales es tendencia en la práctica médica actual. Esta Guía de Práctica Clínica se elaboró respondiendo a la necesidad de protocolizar la atención médico-quirúrgica de la atresia esofágica. En el Centro Territorial de Cirugía Neonatal de Holguín, donde se regionaliza la atención a neonatos de las cinco provincias orientales del país con afecciones congénitas y quirúrgicas de alta complejidad, la atresia esofágica fue la afección quirúrgica más frecuente en los últimos diez años, con una supervivencia ascendente que alcanzó 94,4 por ciento en 2019. La guía que se presenta se aprobó en el Primer Consenso Nacional de Guías de Prácticas Clínicas en Cirugía Pediátrica, en Varadero, Matanzas en 2019. Incluye las principales pautas para el diagnóstico, tratamiento y seguimiento de los pacientes afectados y se considera una herramienta eficiente para mejorar los resultados en la asistencia médica y quirúrgica neonatal(AU)

Esophageal atresia is one of the most common congenital anomalies in neonatal surgical practice. It is estimated to have an incidence of 1 per 3500 live newborns globally. The preparation of action guides and care protocols is a trend in current medical practice. This Clinical Practice Guide was prepared in response to the need to protocolize the medical-surgical care of esophageal atresia. In the Territorial Center for Neonatal Surgery of Holguín, where the care of neonates from the five eastern provinces of the country with congenital and surgical conditions of high complexity is regionalized, esophageal atresia was the most frequent surgical condition in the last ten years, with an ascending survival that reached 94.4 percent in 2019. The guideline presented was approved in the First National Consensus of Clinical Practice Guidelines in Pediatric Surgery, in Varadero, Matanzas in 2019. It includes the main guidelines for the diagnosis, treatment and follow-up of affected patients and is considered an efficient tool to improve outcomes in neonatal medical and surgical care(AU)

Predictors of Mortality after Primary Discharge from Hospital in Patients with Esophageal Atresia.

OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.

Quality of life assessment in esophageal atresia patients: a systematic review focusing on long-gap esophageal atresia.

BACKGROUND: Children born with esophageal atresia (EA) have inherent abnormalities in esophageal motility which may impact upon patient and family Quality of Life (QoL). Currently, paucity of data exists for long-term outcomes of long-gap EA. We aimed to: (1) summarize QoL tools reported in the literature, focusing upon studies involving long-gap EA patients, and (2) compare QoL for long-gap versus non-long-gap EA patients. METHOD: We performed a systematic review of Cochrane Register of Controlled Trials, PubMed, EMBASE, and Ovid databases (January 1980-May 2018) in accordance with the PRISMA protocol. RESULT: Six studies were identified (536 patients total), and 419/536 (78%) patients completed QoL assessment. Response rates ranged from 29% to 100%. Median study size was 86 (range 8-159). Esophageal atresia type was described in 477 patients, and 74/477 (16%) were long-gap. Common assessment tools were Gastrointestinal Quality of Life Index and 36-Item Short-Form Health Survey. Compared with healthy individuals, long-gap EA patients suffered more gastrointestinal symptoms. There were no significant differences in QoL outcomes between long-gap and non-long-gap EA patients. CONCLUSION: Current literature suggests no significant difference in QoL outcomes between long-gap and non-long-gap EA patients. However, due to questionnaire variability and range of response rates, the data should be interpreted with care. LEVEL OF EVIDENCE: Level II.

Newly discovered variant of oesophageal atresia: 'Type Y'.

Multiple variations of oesophageal atresia (OA) have been described. We present two cases of a new variant of OA ('Type Y') where the fistula enters the trachea in a Y-shaped configuration. Awareness of this is important. Bronchoscopy will reveal a single fistula opening and therefore there will initially be no suspicion of anatomical variation. It may be that only one bifurcation of the 'Y' fistula is patent which poses a risk of incomplete fistula closure.

Predictors of the Performance of Early Antireflux Surgery in Esophageal Atresia.

OBJECTIVE: To identify predictors of and factors associated with the performance of antireflux surgery during the first year of life in children born with esophageal atresia. STUDY DESIGN: All patients were included in a French registry for esophageal atresia. All 38 multidisciplinary French centers completed questionnaires about perinatal characteristics and one-year outcome for children born with esophageal atresia. RESULTS: Of 835 infants with esophageal atresia born in France from 2010 to 2014, 682 patients, excluding those with long-gap esophageal atresia, were included. Three patients had type I, 669 had type III, and 10 had type IV esophageal atresia. Fifty-three children (7.8%) received fundoplication during the first year of life. The median age at the time of the end-to-end esophageal anastomosis was 1.1 day (range 0-15). Multivariate analysis identified three perioperative factors that predicted the need for early antireflux surgery: anastomotic tension (P = .004), associated malformations (P = .019), and low birth weight (P = .018). Six other factors, measured during the first year of life, were associated with the need for antireflux surgery: gastroesophageal reflux (P < .001), anastomotic stricture (P < .001), gastrostomy (P < .001), acute life-threatening event (P = .002), respiratory complications (P = .045), and poor nutritional status (P < .001). CONCLUSIONS: Gastroesophageal reflux disease, low birth weight, poor nutrition, and surgical anastomosis difficulties predicted the performance of antireflux surgery in the first year of life in infants with esophageal atresia.

[Oesophageal Atresia - from the Challenge in Prenatal Medicine until Surgical Care]. / Die Ösophagusatresie ­ von der Herausforderung in der Pränatalmedizin bis zur chirurgischen Versorgung.

Oesophageal atresia causes a dysplasia of the oesophagus with or without a connection to the adjoining trachea. Prenatal ultrasound results are not specific enough to confirm a suspected diagnosis. In addition to polyhydramnios and a small or absent stomach, the so-called "pouch sign" reinforces the suspected diagnosis. An MRI increases the prenatal detection rate. Due to the lack of reliable sonografic markers, ultrasonic testing is advised during pregnancy. Particularly, further causes for the polyhydramnios should be categorically excluded. Postnatally, children present with classic symptoms. Surgical treatment results in a very high quality of life and a very good prognosis. Nevertheless lifelong monitoring and follow-up of the patient is required.

Pneumonia after Repair of Esophageal Atresia-Incidence and Main Risk Factors.

INTRODUCTION: Esophageal atresia (EA) is associated with significant respiratory mortality. We aimed to assess incidence and predictive factors of EA associated pneumonia during the first 5 years of life. MATERIALS AND METHODS: Institutional ethical consent was obtained. Hospital records of patients with EA from 2002 to 2017 were reviewed. Episodes of pneumonia that were diagnosed in university or regional hospitals were included. For instance, respiratory infections other than pneumonia, anastomotic complications, aortopexy, fundoplication, major EA associated diseases, types of EA, and EA anastomosis dilatations were tested as potential risk factors for pneumonia. RESULTS: A total of 104 patients (56 males; type A 7, B 3, C 83, D 3, E 6, F 2) was included. Thirty-five (34%) patients had 94 episodes of pneumonia corresponding to median 2 (IQR [interquartile range]: 1-4) and 609 episodes per thousand patient years. Majority of pneumonias occurred before the age of 3 years. The cause of pneumonias could be identified as RS (respiratory syncytial) virus in 15(16%) and aspiration in seven (7.4%) episodes. In univariate analysis, pneumonia was predicted by occurrence and number of nonpneumonia respiratory infections, anastomotic reoperations, fundoplication, and number of EA anastomosis dilatations. In multivariate logistic regression analysis, significant risk factors for pneumonia were occurrence of nonpneumonia respiratory infections and number of anastomotic dilatations. CONCLUSION: Episodes of pneumonia occurred in one-third of patients with EA. After the final repair, the incidence of pneumonia was highest during the first 3 years of life. Patients with other acute respiratory infections and high number of dilatations were at the greatest risk.

Esophageal Atresia with or without Tracheoesophageal Fistula (EA/TEF): Association of Different EA/TEF Subtypes with Specific Co-occurring Congenital Anomalies and Implications for Diagnostic Workup.

BACKGROUND: Esophageal atresia with or without tracheoesophageal fistula (EA/TEF) represents the most common developmental malformation of the upper digestive tract. It is classified into six subtypes according to the classification of Vogt, depending on anatomical variation of this malformation. Around 50% of the patients with EA/TEF present additional anomalies, which often influence, next to the EA/TEF subtype, the overall prognosis of EA/TEF newborns. Here, we investigated the association of the different EA/TEF subtypes with co-occurring congenital anomalies in EA/TEF patients and demonstrate their implications for postnatal diagnostic workup. MATERIALS AND METHODS: We investigated 333 patients of a large German multicenter study born between 1980 and 2012. After evaluation of all available clinical records, 235 patients were included in our analysis. We compared our results with existing data. RESULTS: The highest risk for co-occurring anomalies was seen in patients with most common Vogt 3b (p = 0.024), especially for additional gastrointestinal anomalies (p = 0.04). Co-occurring anomalies of the skin were significantly more common in patients with subtype Vogt 2 (p = 0.024). A significant correlation was observed for an impaired neurodevelopmental outcome and EA/TEF Vogt 3a (p = 0.041). Patients with EA/TEF showed a higher risk to present with any additional congenital anomaly compared with the general population (p < 0.001). CONCLUSION: Our results warrant thorough clinical workup for gastrointestinal anomalies especially in patients with Vogt 3b. Moreover, it might be necessary to focus on a thorough aftercare for neurocognitive development in patients with Vogt 3a. The here presented observations need to be confirmed by future studies.

Mortality and morbidity in oesophageal atresia.

BACKGROUND: Several classification systems exist to predict mortality in oesophageal atresia, the most widely quoted of these being over 20 years old. No classification system exists to predict morbidity. We sought to test whether these classification systems remain relevant and to determine whether they can be useful to predict morbidity. In addition, we aimed to identify independent risk factors for predicting mortality and morbidity. METHODS: Neonates presenting with oesophageal atresia over a 20-year period (1990-2010) were retrospectively reviewed. Discriminative statistical analysis compared the performance of current classification systems. Stepwise logistic regression analysis of the influence of perioperative risk factors on mortality and duration of ventilatory support and intensive care unit stay were performed. RESULTS: All classification systems predicted mortality in this series of 248 neonates. Birth weight, cardiac anomalies and pre-operative pneumonia were independent risk factors for predicting mortality in oesophageal atresia. The Waterston classification is the most useful classification for predicting post-operative morbidity in terms of length of hospital stay and time spent ventilated. CONCLUSION: Despite advances in the neonatal care of the very low birth weight infant and those with congenital cardiac disease, these conditions remain relevant in predicting mortality and morbidity in oesophageal atresia.

Long-term morbidity in adolescents and young adults with surgically treated esophageal atresia.

PURPOSE: To investigate the long-term morbidity of surgically treated esophageal atresia (EA) in adolescents and young adults and establish whether these long-term morbidities are affected by the type of EA. PATIENTS AND METHODS: We reviewed the medical records, including backgrounds and associated conditions, of 69 long-term survivors of EA, aged >15 years. The long-term morbidities included neurodevelopmental abnormality, nutritional impairment (short height <-2SD, low BMI <18.5), subjective symptoms, and musculoskeletal deformities. Comparisons of the results were made between Gross A-type EA (n = 6) and Gross C-type EA (n = 63). RESULTS: All patients underwent esophageal anastomosis without esophageal replacement. Cardiac anomalies and long gap were present in 26 and 18%, respectively. Esophageal dilatation, fundoplication, and aortopexy were performed in 40, 34, and 18%, respectively. The incidence of long gap and esophageal stenosis was higher in Gross A-type EA than in Gross C-type EA. The long-term morbidities included neurodevelopmental abnormality (13%), nutritional impairment (62%: as short height in 34% and as low BMI in 46%), subjective symptoms (14%), and musculoskeletal deformities (59%). There were no differences in the long-term morbidities between Gross A and Gross C. CONCLUSIONS: The incidence of the long-term morbidities is high in adolescents and young adults, regardless of the type of EA. Early detection of morbidity is important to improve the long-term outcomes of EA.

Long-Gap Esophageal Atresia Is a Unique Entity within the Esophageal Atresia Defect Spectrum.

BACKGROUND: Long-gap esophageal atresia (LGEA) may have clinical and syndromic presentations different from those of esophageal atresia (EA) that affects shorter segments of the esophagus (non-LGEA). This may suggest unique underlying developmental mechanisms. OBJECTIVES: We sought to characterize clinical differences between LGEA and non-LGEA by carefully phenotyping a cohort of EA patients, and furthermore to assess molecular genetic findings in a subset of them. METHODS: This is a retrospective cohort study to systematically evaluate clinical and genetic findings in EA infants who presented at our institution over a period of 10 years (2005-2015). RESULTS: Two hundred twenty-nine EA patients were identified, 69 (30%) of whom had LGEA. Tracheoesophageal fistula was present in most non-LGEA patients (158 of 160) but in only 30% of LGEA patients. The VACTERL association was more commonly seen with non-LGEA compared to LGEA (70 vs. 25%; p < 0.001). Further, trisomy 21 was more common in LGEA than in non-LGEA. 25% of LGEA patients had an isolated EA diagnosis without other anomalies, compared to <1% for non-LGEA. Chromosomal microarray analysis showed copy number variations (CNV) in 4 of 39 non-LGEA patients and 0 of 3 LGEA patients. A review of the ClinGen database showed that none of those CNV have been previously described with EA. CONCLUSIONS: LGEA represents a unique type of EA. Compared to non-LGEA, it is more likely to be an isolated defect and associated with trisomy 21. Further, it is less commonly seen with VACTERL anomalies. Our findings suggest the involvement of unique pathways that may be distinct from those causing non-LGEA.

Esophageal Atresia: Improved Outcome in High-Risk Groups Revisited.

Objectives Improved survival in infants with esophageal atresia (EA) with a birth weight < 1,500 g or a major cardiac anomaly has been reported when compared with the original Spitz classification proposed in 1994. Aim We reviewed outcome data for infants born over the last decade in our institution to update previously reported survival statistics. Materials and Methods The records of all neonates (n = 200) with a diagnosis of EA managed in a single institution between 2001 and 2011 were reviewed and compared with data from the original Spitz study and the subsequent reported cohort from the same institution. Data were obtained on birth weight, presence of a major cardiac anomaly, and survival. Differences in survival were compared using the Yates-corrected chi-square test. Local ethical study approval was obtained. Results Infants born over the last decade had a comparable overall survival rate of 93% (186/200) versus 92.6% (174/188) in the previously reported cohort (1993-2004). We demonstrate an improved survival as compared to the Spitz cohort (87.6%, 326 /372, p = 0.06) and a statistically significant improvement in survival in Group II (p = 0.01). Within this group, 12/51 neonates had a birth weight < 1,500 g and 39/51 had major cardiac anomalies. Of interest, of the nine deaths in Group II, eight were in the subgroup with major cardiac anomalies. Conclusion The survival of neonates in Group II has significantly improved. Mortalities within this group were predominantly in the subgroup with major cardiac anomalies suggesting birth weight is of less significance than in previous years reflecting recent advances in neonatal care. We propose an updated prognostic classification that makes a distinction between cardiac and low-birth-weight infants.

[Does prenatal diagnosis modify neonatal management and early outcome of children with esophageal atresia type III?]. / Le diagnostic anténatal modifie-t-il la prise en charge néonatale et le devenir à 1 an des enfants suivis pour atrésie de l'œsophage de type III ?

OBJECTIVE: Evaluate neonatal management and outcome of neonates with either a prenatal or a post-natal diagnosis of EA type III. STUDY DESIGN: Population-based study using data from the French National Register for EA from 2008 to 2010. We compared children with prenatal versus post-natal diagnosis in regards to prenatal, maternal and neonatal characteristics. We define a composite variable of morbidity (anastomotic esophageal leaks, recurrent fistula, stenosis) and mortality at 1 year. RESULTS: Four hundred and eight live births with EA type III were recorded with a prenatal diagnosis rate of 18.1%. Transfer after birth was lower in prenatal subset (32.4% versus 81.5%, P<0.001). Delay between birth and first intervention was not significantly different. Defect size (2cm vs 1.4cm, P<0.001), gastrostomy (21.6% versus 8.7%, P<0.001) and length in neonatal unit care were higher in prenatal subset (47.9 days versus 33.6 days, P<0.001). The composite variables were higher in prenatal diagnosis subset (38.7% vs 26.1%, P=0.044). CONCLUSION: Despite the excellent survival rate of EA, cases with antenatal detection have a higher morbidity related to the EA type (longer gap). Even if it does not modify neonatal management and 1-year outcome, prenatal diagnosis allows antenatal parental counseling and avoids post-natal transfer.

[Recurrent tracheoesophageal fistula in type III esophageal atresia. Diagnosis and treatment are not easy]. / Reperméation de fistule œso-trachéale dans l'atrésie de l'œsophage de type III : un diagnostic et une prise en charge difficile.

Recurrent tracheoesophageal fistula (TEF) is possible after repair of congenital esophageal atresia. The frequency of recurrent TEF is observed in about 10% of the cases. Within a cohort of 67 children with type III esophageal atresia repaired between 1998 and 2009, we aimed to identify the number of children with recurrent TEF, the risk factors for this condition, and the treatment proposed. The sex ratio was 1.7. Surgery was performed between 4 and 36 hours of life. Five children (7.5%) had a recurrent TEF, usually during the first 3 months, revealed by respiratory symptoms related to feeding in some cases. We noted that recurrent TEF was more frequent with anastomotic leakage (P=0.09) or postsurgical pneumothorax (P<0.01). The diagnosis was made in four cases out of five by a methylene blue test performed during a tracheobronchial endoscopy. Surgical treatment was noted in four children, with three postsurgical secondary effects. One child was treated by endoscopy and an esophageal clip. With a median follow-up of 52 months, no recurrence was noted. The recurrence of TEF may be linked to postsurgical events. Diagnosis is not easy and treatment is not clearly codified. Endoscopic treatment may be an advantageous option to surgery, likely with less morbidity.

[Medium-term outcome, follow-up, and quality of life in children treated for type III esophageal atresia]. / État de santé, suivi et qualité de vie à moyen terme d'enfants opérés d'une atrésie de l'œsophage de type III.

The aim of this study was to evaluate the medium-term outcome (health status, medical and surgical French National Health Authority-recommended follow-up, and quality of life) of children born with type III esophageal atresia (EA). Previous events (during the perinatal period, associated abnormalities, respiratory and digestive complications) of children treated for type III EA at the Marseille university hospitals between 1999 and 2009 were noted. Parents completed a standardized questionnaire concerning the health of their children during the previous year, and a quality-of-life questionnaire (PedsQL 4.0) was also completed by children aged more than 8 years. Among the 68 children treated, 44 responded to our solicitation (mean age, 7.6 years; range, 3-12.8 years). Previous important events were : pneumonia(s) (65%), asthma before the age of 3 years (66%), hospitalization for a respiratory event (45%), fundoplication (20%), and esophageal dilatation (45%). We noted current chronic cough (16%), asthma (30%), dysphagia (39%), and symptomatic gastroesophageal reflux (9%). National guidelines were not respected, except for the surgical indications in children aged less than 6 years. The quality-of-life scores (n=43 children) were similar to healthy controls but were negatively influenced by a gastrostomy procedure (P=0.020), pneumonia (P=0.013), and hospitalization due to a respiratory event (P=0.006) or a digestive event (P=0.010), and also by current asthma (P=0.004). In conclusion, despite recurrent respiratory or digestive symptoms and inadequate recommended follow-up, the quality of life of children treated for type III of EA is good.

A case series study of therapeutic implications of type IIIb4: a rare variant of esophageal atresia and distal tracheoesophageal fistula.

BACKGROUND: A wide spectrum of variations can occur in type IIIb esophageal atresia and distal tracheoesophageal fistula [EA-TEF] (Kluth D. Atlas of esophageal atresia. J Pediatr Surg 1976; 11 (6):901-19). The aim of this study was to evaluate the variant anatomy of subtype IIIb4 EA-TEF and its therapeutic implications. METHODS: We performed a retrospective review of 4 patients of this variant subtype within a series of 259 esophageal atresias, managed over a study period of three years, at our institution. The diagnosis was made at thoracotomy. The overlapping upper and lower esophageal pouches, initially, gave a false impression of esophageal continuity. The long TEF coursed parallel in proximity to the upper pouch (UP) and the trachea needing meticulous dissection. The TEF measured 8-10mm in diameter and was ligated and divided high on the trachea near the thoracic inlet. The patients were followed up by a contrast swallow, radioactive technetium-99m- sulfur colloid scan, echocardiography and bronchoscopy (if indicated). RESULTS: The mean birth weight and gestational age was 2250 g and 38 weeks respectively. The mean follow up was eighteen months (range 5-42 months). An associated anorectal malformation was present in two patients while none had associated congenital heart disease. The Waterston grading was A and B in one patient each and C in two patients. All the patients had muscular UP extending up-to the level of arch of azygous vein (T(3-4)). The lower esophageal pouch (LP) and the fistula extended high, near the thoracic inlet. Tracheal injury occurred in one patient, with thin LP, warranting postoperative elective ventilation. A postoperative barium swallow did not reveal anastomotic leak or stricture in any patient. Follow-up barium swallow showed long LP extending high up in the chest, up to clavicles in all four patients and redundancy of UP in case 1. Severe gastroesophageal reflux was present in two patients. Bronchoscopy revealed tracheomalacia in case 1. Three patients were thriving well at the last follow-up. One patient died at five months of age due to aspiration pneumonia. CONCLUSIONS: The knowledge of diagnostic and therapeutic implications of the variant subtype IIIb4 EA-TEF should be in the armamentarium of the treating surgeon to reduce pitfalls in its management.

Clinical outcomes of esophageal atresia: comparison between the Waterston and the Spitz classifications.

INTRODUCTION: Preoperative prognostic predictors are important for surgeons and parents to estimate the survival of patients with esophageal atresia (EA). The aim of this study was to update the clinical outcomes of EA treatment by comparing between the Waterston and the Spitz classification. MATERIALS AND METHODS: Medical records of the patients with EA treated at Queen Sirikit National Institute of Child Heath from 2003 to 2010 were reviewed. All of the patients were categorised into 3 groups of the Waterston and 3 groups of the Spitz risk factor criteria for comparing of the differences in each group and each classification. RESULTS: One hundred and thirty-two patients (81 males and 61 females) were treated for EA during the study period. Applying the Waterston classification, survival rate was 100% in group A, 91.5% in group B and 48.8% in group C. There was no statistical difference between the survival rate in group A and group B (P = 0.119) but significant difference between group B and group C (P = 0.000). Using the Spitz classification, survival rate was 97.4% in group I, 64.4% in group II and 27.3% in group III. There was obviously statistical difference of the survival rate between each group (group I vs group II, P = 0.000; group II vs group III, P = 0.041). CONCLUSION: Comparing with the prognostic predictors, the Spitz classification was more valid than the Waterston criteria. The Spitz classification is suitable to use for preoperative predictor to parental counselling and comparing of treatment outcomes of EA among paediatric tertiary care centres.

Long-gap esophageal atresia: traction-growth and anastomosis - before and beyond.

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.

Congenital bronchopulmonary foregut malformation initially diagnosed as esophageal atresia type C: challenging diagnosis and treatment.

Communicating bronchopulmonary foregut malformations are extremely rare congenital malformations, characterized by a communicating fistula between an isolated part of the respiratory system and the esophagus or the stomach. In this article, we present a case of esophageal atresia type C, later diagnosed as a rare form of a communicating bronchopulmonary foregut malformation, an esophageal atresia combined with right main bronchus originating from the lower esophagus. Therapeutic resection of the right lung was complicated by postpneumonectomy syndrome.