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Multicentre survey on the current surgical management of oesophageal atresia in Belgium and Luxembourg
Journal of Pediatric Surgery 52 (2017) 239–246
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Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Multicentre survey on the current surgical management of oesophageal atresia in Belgium and Luxembourg Helena Reusens ⁎,1, Lucas Matthyssens 1, Charlotte Vercauteren, Katrien van Renterghem, and the members of the Belgian Association of Paediatric Surgery (BELAPS) 2 Department of Paediatric Surgery/Gastro-intestinal Surgery, Princess Elisabeth Children's Hospital, Ghent University Hospital, Ghent, Belgium
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Article history: Received 31 October 2016 Accepted 8 November 2016 Key words: Oesophageal atresia Tracheoesophageal fistula Questionnaire Survey Practice
a b s t r a c t Introduction: The surgical management of oesophageal atresia (OA) differs between pediatric surgical teams without consensus. We aimed to describe the current practice of OA treatment in Belgium and Luxembourg and compare this to the literature. Materials and methods: A questionnaire was created and sent to all 18 hospitals (14 pediatric surgical units) performing OA surgery in Belgium and Luxembourg. The results were compared to the literature. Results: Most units treat an average of 2–5 OA + TOF (71%) and ≤1 pure OA (pOA) per year (86%). The preferred surgical approach for OA + TOF is thoracotomy (86%), mostly extra-pleural (75%). Thoracoscopic OA repair is performed in 21%. All centers perform an end-to-end anastomosis (interrupted sutures), and all leave a transanastomotic tube. A chest drain is routinely used in 8 units (57%). In pOA the preferred surgical approach is gastrostomy formation with delayed primary anastomosis (77%). The timing for delayed anastomosis is 2 to 24 months. Intra-operative lengthening is mostly attempted with Foker technique (46%). If oesophageal replacement is needed, gastric interposition is mostly used (75%). A postoperative contrast study is routinely performed in 86% for OA + TOF and in 100% for pOA. Anti-reflux medication is routinely prescribed by all units but one. Conclusion: There are still many differences and controversies in the perioperative management of OA. Part of this is based on habits and is difficult to change without scientific evidence. There is a need for prospective (inter)national registries to further identify the existing differences, leading to a more widely accepted consensus. Level of Evidence: Level III. © 2017 Elsevier Inc. All rights reserved.
Oesophageal atresia (OA) is a rare congenital malformation characterized by an anatomical discontinuity of the oesophagus with or without tracheoesophageal fistula (TOF). The prevalence of OA in reported series varies from 1.2 to 4.5 per 10.000 live births, with a TOF being present in 72% to 94% [1–2]. Many neonates with OA present with other associated anomalies or a genetic syndrome [3]. Their diagnosis is essential to determine the correct medical and surgical management of OA [4]. Since the first attempt to correct OA in 1888, there has been a continuing evolution in surgical techniques, neonatal and anesthetic care, with important impact on survival rates. Current survival rates are around 90%, with almost 100% survival in term neonates without associated anomalies [1,2,4].
⁎ Corresponding author at: Université Libre de Bruxelles (ULB), Department of Paediatric Surgery, Hôpital Universitaire des Enfants Reine Fabiola, Avenue Crocq 15, 1020, Brussels, Belgium. Tel.: +32 496 119 705; fax: +32 2477 34 49. E-mail addresses: [email protected], [email protected] (H. Reusens). 1 Joint first authors. 2 Additional co-authors, names and institutions are cited under Acknowledgements. http://dx.doi.org/10.1016/j.jpedsurg.2016.11.010 0022-3468/© 2017 Elsevier Inc. All rights reserved.
Many steps in the surgical management of OA remain however controversial. Recent (inter)national surveys and cohort studies assessing surgical management strategies of OA have confirmed persisting differences in practice and habits [2,5–7]. The aim of this study was to obtain a complete overview of the actual surgical management of OA amongst all pediatric surgical teams in Belgium and Luxembourg, to describe the differences and variations and compare this to the available literature.
1. Materials and methods Based on the literature, a novel questionnaire on the perioperative management of OA was constructed (Fig. 1). Three independent Belgian pediatric surgeons, board members of the Belgian Association of Pediatric Surgery (BELAPS), reviewed it and could add suggestions. It contained questions concerning prevalence, preoperative investigations, surgical techniques, postoperative management, anti-reflux medication and follow-up. A distinction was made between OA + TOF and pure OA (pOA). The questionnaire was sent to all 18 centers offering pediatric surgical care for OA in Belgium and Luxembourg, to be completed by
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the head of the department or by the team-member with specific interest/ expertise in this topic. Anonymity was guaranteed.
larger ‘Pediatric Surgical Unit’ (PSU) using the same management protocols, 14 PSU were identified for evaluation. 2.2. Cited local OA prevalence and team composition (Table 1)
2. Results 2.1. Survey participation All pediatric surgical teams in Belgium and Luxembourg responded. Since some teams and hospitals replied to be working together as one
Ten PSU (71%) replied to be treating ‘2 to 5 OA + TOF’ per year. Two PSU treat ‘less than 2 OA + TOF’ per year, and two PSU treat ‘between 5 and 10 OA + TOF’ per year. Concerning pOA, 13 of the 14 PSU replied to surgically treat pOA. Most PSU replied to perform ≤1 procedure per year (n = 12, 85%). Two PSU (15%) replied to perform ‘2–4 cases’ per year. In
Fig. 1. Questionnaire used for this survey.
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Fig. 1. (Continue)
Belgium and Luxembourg, there are 30 surgeons treating OA in 14 PSU, with a median of 2 surgeons per PSU (range 1–6).
None of the PSU are performing preoperative contrast studies, CT or MRI. Genetic screening is performed routinely in 4 PSU (29%).
2.3. Preoperative investigations 2.4. Surgical technique in OA + TOF Routine echocardiography is performed preoperatively in all centers for OA + TOF and pOA. A flexible tracheobronchoscopy is routinely performed by 5 PSU (36%) for OA + TOF and by 6 PSU (46%) for pOA. Oesophagoscopy is routinely performed in 1 PSU in case of pOA (7%).
The preferred surgical approach for OA + TOF is thoracotomy (n = 12, 86%) with posterolateral muscle-sparing technique (n = 7, 58%) and extrapleural approach (n = 9, 75%). Thoracoscopy is
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Fig. 1. (Continue)
performed in 3 PSU (21%). In 6 PSU (43%) the azygos vein is preserved during dissection. Intraoperative measurement of the oesophageal gap in case of OA + TOF is routinely performed in 6 PSU (43%): before dissection in 33% (n = 2), before and after dissection in 66% (n = 4). Different methods are used (string, vesselloop, instrument). For pOA, the gap length is routinely measured in all PSU. All PSU perform an end-to-end anastomosis with interrupted sutures. The sutures used are absorbable in 93% (n = 13). Closure of the TOF is mostly performed with absorbable sutures (n = 8, 57%). All
PSU leave an oesophageal transanastomotic tube (TAT). A chest drain is routinely placed in 57% (n = 8). 2.5. Pure and ‘long gap’ oesophageal atresia 2.5.1. Definition of ‘long gap’ The measurement of the gap is expressed in vertebral bodies (VB) in 92% (n = 12) and in centimeters (cm) in 23% (n = 3), one PSU uses both VB and cm. The definition of ‘long gap’ (LG) is controversial. Six PSU (46%) define LG to be a distance of ‘more than 3 VB’. One of these
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Fig. 1. (Continue)
also uses a measurement of ‘more than 3 cm’ as a cut-off value. In two PSU (15%) LG is defined as ‘more than 2 VB’, and one of these equals this with ‘more than 2 cm’ distance. Five PSU (38%) only use ‘impossible primary anastomosis’ to define LG. Overall, ‘impossible primary anastomosis’ is for 8 PSU (62%) an acceptable definition for LG. 2.5.2. Surgical approach Pure OA is treated in 13 of the 14 PSU (93%). The preferred surgical approach is delayed primary anastomosis (DPA) with gastrostomy formation without oesophagostomy in 10 PSU (77%). One PSU (8%) attempts a primary anastomosis without gastrostomy or oesophagostomy. Two PSU (15%) perform DPA with routine gastroand oesophagostomy. The median delay for DPA is 5 months (range 2–24). Four PSU (31%) perform a routine thoracoscopic repair, but most favor a thoracotomy (n = 9, 69%). Preoperative elongation techniques are performed in 6 PSU (46%), mainly by serial bougienage (n = 5, 83%). Intra-operative lengthening is attempted by 7 PSU (54%), mainly by the Foker technique (n = 6, 86%)
or by a spiral myotomy (n = 2, 28%). In one PSU flap oesophagoplasty has been used. There is a full consensus to leave a TAT. A chest drain is routinely placed in 77% (n = 10). If necessary, oesophageal replacement is performed in 61% of PSU (n = 8), with a preference for gastric interposition (n = 6, 75%). In 1 PSU (12%) colonic interposition is performed as a standard technique for all pOA and LG. In another PSU both gastric and colonic interpositions are performed.
2.6. Postoperative management Routine prolonged ventilation is used in 7 PSU (50%) in OA + TOF and in 8 PSU (61%) in pOA, mainly to reduce tension on the anastomosis (57%–62%) or for respiratory reasons (43%–37%). This paralysis is continued for an average of 2.3 days (range 2h-5d). Routine head flexion is used in 1 PSU (8%) in OA + TOF and in 4 PSU (31%) in pOA.
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Table 1 Overview of surveys and large prospective cohort studies on OA. SURVEYS
Number of participants Prevalence OA/TOF: 2–5/year * 5–10/year
PROSPECTIVE COHORTS
Current report
Zani 2014
Shawyer 2014
Lal 2013
Ron 2009 (long gap)
Burge 2013
Sfeir 2013
Pini Prato 2015
14 PSU 30 surgeons
160 surgeons
57 surgeons
170 surgeons
69 surgeons
28 PSU
38 PSU
52 PSU
71% 14%
31% 36%
b5/center 26% 21%
1–3/surgeon 67% 4–7/surgeon 24%
Prevalence pOA: 3–5/surgeon 20% N5/surgeon 7%
Prevalence OA: x x x x
50% N5/center 15%
PSU: Pediatric Surgical Unit; OA/TOF: Oesophageal Atresia with Tracheoesophageal Fistula; pOA: pure Oesophageal Atresia.
Routine contrast study is performed by 86% (n = 12) in OA + TOF and in 100% (n = 13) in pOA at a median of 6 postoperative days (range 3–10). This is reflected in the start of oral feeding after postoperative day (POD) 5 in 12 PSU (85%). In one PSU oral feeding is started without contrast study on POD 1 or 2. Enteral feeding through the TAT is administered in 92% (n = 13), starting on POD 3 to 4 in 57%, and starting before POD 2 in 14%. When pOA, all PSU first perform contrast study before starting oral feeding after POD 5. In these patients, enteral feeding (TAT) is administered in 38% (n = 5) at POD 3–4 and in 46% (n = 6) after POD 5.
2.7. Postoperative anti-reflux medication and follow-up Thirteen PSU (93%) routinely prescribe anti-reflux medication postoperatively. The duration of the medical treatment varies: three months (n = 3, 23%), six months (n = 2, 15%) to 12 months (n = 7, 54%). In one PSU, the duration depends upon findings at routine oesophagogastroduodenoscopy (OGD). Routine postoperative OGD is performed in 3 PSU (21%) for OA/TOF and in 4 PSU (31%) for pOA. The duration of postoperative follow-up varies: ‘less than 1 year’ in one PSU (7%), ‘between 1 and 5 years’ in three PSU (21%), ‘between 5 and 10 years’ in three PSU (21%), ‘between 10 and 16 years’ in two PSU (14%) and ‘more than 16 years’ in five PSU (37%).
3. Discussion This survey is the result of the voluntary joint effort of all pediatric surgical units in Belgium and Luxembourg. It reflects the current perioperative management of OA, based upon a complete (100%) response rate.
3.1. Prevalence/Center volume (Table 1) A bit of caution is needed when interpreting the prevalence and center volume numbers in this survey: all numbers were cited by the centers themselves and not further verified. In larger centers, there may be more than one surgeon performing OA surgery, which may decrease the number of procedures per individual surgeon. In small centers all procedures will possibly be performed by only one surgeon. In this survey, 50% of the teams reported that only a selected number of surgeons perform surgery for OA. Only a few studies on OA repair report on volumeoutcome results. A recent large cohort study in France showed no significant correlation of caseload per center and survival, fistula recurrence, anastomotic stenosis, or oesophageal replacement rates [2]. In contrast, a recent study on thoracoscopic OA repair in Japan showed that all anastomotic leakages occurred in ‘high volume’ centers (N10 OA/year) [7]. The question remains whether concentrating congenital oesophageal pathology in high volume centers or dedicating pediatric surgeons subspecialised in foregut pathology, or a combination of both, will offer a beneficial effect on the postoperative outcomes of OA repair.
3.2. Preoperative investigations (Table 2) Although consensus exists on the routine preoperative use of cardiac ultrasonography in this survey, the use of routine tracheobronchoscopy is still controversial (36–46%), corresponding to the literature (range 21,5–60%) [2,6,8,9]. More than 50% of patients with OA have associated anomalies, more than 20% are born with a VACTERL association or genetic syndrome [1,3,6,10]. Nonetheless, only 4 PSU (30%) in this survey perform a genetic work-up. This number is fairly low, but its interpretation remains difficult as this question wasn't included in other published studies.
Table 2 Overview of results in surveys and large prospective cohort studies on OA. SURVEYS
PROSPECTIVE COHORTS
Current report
Zani 2014
Shawyer 2014
Lal 2013
Burge 2013
Sfeir 2013
Pini Prato 2015
Pre-op echocardiogram Pre-op bronchoscopy
100% 36% OA + TOF 46% pOA
81% 43%
x x
83.2% x
x 21.5%
98.6% 47%
Routine use thoracoscopy
21% OA + 31% pOA 57% OA + 77% pOA 100% 50% OA + 61% pOA 86% OA + 100% pOA 93%
TOF
6%
x
x 60% routine 13% selective 50%
2.5%
x
3%
TOF
69%
70%
83%
53.7%
x
91%
TOF
90% 56%
85% x
84% 25%
98.3% 89,5%
x x
88% x
TOF
72%
70%
85%
36.3%
x
84%
x
86%
76%
51.6
86%
x
Chest drain TAT Prolonged ventilation Contrast study Reflux medication
OA + TOF: oesophageal atresia with tracheoesophageal fistula; pOA: pure oesophageal atresia; TAT: transanastomotic tube; x: no data available.
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3.3. Operative approach
3.4. Postoperative management
3.3.1. Thoracoscopic approach A recent meta-analysis confirmed the effectivity and safety of thoracoscopic OA repair with comparable outcomes to conventional open repair [11]. In our survey, 21% of PSU perform thoracoscopic repair. Although neonatal thoracoscopy becomes increasingly popular [9,12], the amount of centers routinely performing it for OA remains low (Table 2), e.g. only 6% in the EUPSA-BAPS survey [8]. In contrast, the IPEG survey showed thoracoscopic repair in 50% [9], which can be explained by the IPEG members' keen interest in minimally invasive techniques. Other studies mention a lower incidence or do not mention thoracoscopy at all [2,5,6].
3.4.1. Contrast study The use of routine oesophageal contrast studies before the initiation of oral feeding, has since long been debated [18–20]. In Belgium and Luxembourg 86% of PSU does a routine contrast study (100% after pOA repair). In other studies this ranges from 70 to 85% (Table 2). The only exception is the UK, where only 36.3% perform a postoperative contrast study [5]. The authors state that routine contrast study rarely detects a leak and it may delay commencement of feeds and prolong hospital stay.
3.3.2. Surgical technique When attempting a primary oesophageal anastomosis by thoracotomy, the extrapleural approach is the current standard practice in Belgium and Luxembourg (75%). This is comparable to the results in the UK (79%) [5] and Italy (85%) [6]. The type of incision is not fully agreed upon, as approximately 20–25% prefer the axillary incision [6,8,10], above the muscle-sparing posterolateral incision [8]. In this survey, all surgeons agree that the oesophago–oesophageal anastomosis should be performed as an end-to-end anastomosis with interrupted sutures. Most surgeons use absorbable sutures, similar to the IPEG survey [9]. There is also full consensus on leaving a TAT, which is reflected in literature (Table 2). Leaving a chest drain is a topic of ongoing debate [13,14]. A randomized prospective study has shown that a prophylactic extra-pleural chest drain does not decrease early respiratory complications and mortality rates [14]. However, 57% of PSU in the current survey routinely leave a chest tube, which is comparable to other studies (Table 2).
3.3.3. Pure OA The preferred surgical approach in case of pOA is a delayed primary anastomosis with formation of a gastrostomy without oesophagostomy (77%), comparable with 60% of surgeons in the EUPSA-BAPS survey [8] and in agreement with other authors [2,5]. Spitz suggested that “it is generally unproductive to wait longer than 12 weeks in the expectation that the gap will narrow sufficiently to permit a primary anastomosis” [15]. However 23% of PSU wait up to 1 (or 2) years to perform a DPA and two PSU directly opt for oesophageal replacement by colon (at 1 year) or stomach (at 2 months) without attempted elongation or lengthening. In the EUPSA-BAPS survey the delay for DPA ranged between 1 to 12 months (mean 3 months) [8]. In the Italian study the median age of anastomosis was 63 days (range 28–100) [6], which is comparable to the UK (mean 78 days, range 5–135) [5]. Concerning the elongation of the oesophageal ends, there are many different opinions leading to a wide range of surgical habits. Following the ideas of Howard and Foker that “One's own esophagus is best” [12,16], in more than 75% of PSU, techniques are used to attempt delayed primary anastomosis in case of pOA and LGOA: by natural growth, by serial bouginage (39%) with or without gastric bolus feeds, as well as by intraoperative or staged traction if necessary (46%) [5,7,8,15]. Only three PSU (23%) routinely use myotomy or flap reconstruction of the oesophageal ends [5]. When oesophageal replacement is necessary, the majority (70–87%) prefer using the stomach, citing good long-term functional outcome and a satisfied surgeon [2,8,9,12]. This can relate to the ease and reliability of the procedure, as cited by Spitz [8,10,12]. Colonic interposition is only performed by two centers in Belgium and Luxembourg. The use of jejunal interposition in OA patients is rarely first choice for oesophageal replacement [8,9]. A recent meta-analysis failed to show any advantage of one replacement technique over the others [17].
3.4.2. Feeding Although all PSU use a TAT, most PSU (85%) wait with initiating oral feeding until POD 5 or when the contrast oesophagogram shows no leakage. Most PSU will start to administer total parenteral nutrition (TPN) during the first postoperative days. One PSU (7%) starts oral feeding within 24–48 h after surgery without contrast study, a practice also cited by Burge et al. [5], which may shorten the duration of TPN by shortening the times to full enteral and full oral feeding [21]. 3.4.3. Prolonged ventilation Routine prolonged ventilation with paralysis is used by 50% of PSU in this survey, which is comparable with other surveys (Table 2). The median duration is between 2 and 3 days [2,5,8], but may be longer than 7 days in some centers [9]. The main reason for prolonged ventilation is to reduce anastomotic complications by decreasing anastomotic tension [22,23]. Currently, there is little scientific rationale for this technique. The most common indication is actually ‘surgical habit’ or the request by neonatologists, anesthetists or intensivists [5]. 3.4.4. Anti-reflux medication OA is associated with a higher incidence of GORD because of inherent dysmotility of the esophagus and lower oesophageal sphincter [24], or anatomical changes after surgical repair (vagal nerve damage, shortened intra-abdominal esophagus) [25]. GORD may lead to early postoperative complications such as oesophagitis, stricture formation, aspiration pneumonia or failure to thrive [25,26]. In this survey 92% of PSU routinely prescribe anti-reflux medication postoperatively. Comparable to the literature (Table 2), there is however no consensus regarding the use, duration, or type of medication [25]. Some authors recommend anti-reflux medication for at least 6 to 12 months, or even indefinitely, if the patient has symptoms [25,27,28]. 3.4.5. Follow-up In their study comparing 101 adults post OA repair with a control group [29], Rintala et al. described important morbidity in adult OA patients, with long-term problems such as dysphagia, GORD, respiratory symptoms, asthma, infections and also scoliosis and rib cage deformities after open repair. Especially the incidence of GORD in adult survivors of OA is significantly higher than in the general population (33% vs. 10%) [29]. OA survivors may also have a statistical risk of Barrett metaplasia and oesophageal cancer that is (up to 500-fold) greater than that of the general population (although the overall cancer risk seems similar between both groups). These findings indicate the need for a longterm follow-up for all OA patients with an active oesophageal surveillance program. Fifty percent of the PSU in our survey replied to be following the patients for more than ten years, similar to other recent surveys [8], but in need of improvement. 4. Conclusion The results of this survey illustrate that in Belgium and Luxembourg, as in other parts of the world, there is still no general consensus regarding the surgical management of OA.
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(Inter)nationally shared definitions and management guidelines may optimize healthcare by improving patient outcomes and reducing costs. Especially in complex surgical domains, guidelines may improve results, costs and quality of care [9]. In the absence and impossibility of randomized controlled trials for OA, (inter)national multicentric OA registries may provide a more accurate picture of the incidence and outcomes of OA-management. Conflict of interest None. Acknowledgements We sincerely would like to thank all pediatric surgeons in Belgium and Luxembourg and members of the Belgian Association of Pediatric Surgery (BELAPS) who voluntarily cooperated in this international survey. The following pediatric surgeons and BELAPS members, in alphabetical order, are to be acknowledged as co-authors of this paper: Willy Coosemans1, Martine Dassonville2, Antoine De Backer3, Martine Demarche4, Beelke D'hondt5, Marc Dirix6, Paul Leyman7,8, Pierre Lingier9, Paul Philippe10, Anna Poupalou11, Raymond Reding5, Martin Ruppert12, Koenraad Schwagten 7,8, Alexandre Targnion13, Sebastiaan Van Cauwenberge14, Dirk Van de putte15, Erwin Van Der Veken2,16, Erik Van Hoorde17 and Dirk Vervloessem7, 8. 1 University Hospital Gasthuisberg, KUL, Leuven, Belgium; 2 Hôpital Universitaire des Enfants Reine Fabiola, ULB, Brussels, Belgium; 3 Brussels University Hospital, VUB, Brussels, Belgium; 4 CHR de la Citadelle, Liège, Belgium; 5 Cliniques Universitaires St-Luc, UCL, Woluwé, Belgium, 6 CHC Clinique de l'Espérance, Liège, Belgium; 7 AZ St-Augustinus, Antwerp, Belgium; 8 Queen Paola Children's Hospital, ZNA, Antwerp, Belgium; 9 Hôpital Erasme, ULB, Brussels, Belgium; 10 Clinique Pédiatrique, CHL, Luxembourg, Luxembourg; 11 Hôpital Saint-Pierre, ULB, Brussels, Belgium; 12 Antwerp University Hospital, UA, Antwerp, Belgium; 13 CHR de Namur, Namur, Belgium; 14 AZ St-Jan, Bruges, Belgium; 15 Ghent University Hospital, Ghent, Belgium; 16 Hôpital de Jolimont, La Louvière, Belgium; 17 CHU Hôpital Civil Marie Curie, Charleroi, Belgium. References [1] Pedersen RN, Calzolari E, Husby S, et al. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child 2012; 97:227–32. [2] Sfeir R, Bonnard A, Khen-Dunlop N, et al. Esophageal atresia: data from a national cohort. J Pediatr Surg 2013;48:1664–9. [3] Geneviève D, de Pontual L, Amiel J, et al. Genetic factors in isolated and syndromic esophageal atresia. J Pediatr Gastroenterol Nutr 2011;52(Suppl. 1):S6–8. [4] Wang B, Tashiro J, Allan BJ, et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res 2014;190:604–12.
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Journal of Pediatric Surgery journal homepage: www.elsevier.com/locate/jpedsurg
Multicentre survey on the current surgical management of oesophageal atresia in Belgium and Luxembourg Helena Reusens ⁎,1, Lucas Matthyssens 1, Charlotte Vercauteren, Katrien van Renterghem, and the members of the Belgian Association of Paediatric Surgery (BELAPS) 2 Department of Paediatric Surgery/Gastro-intestinal Surgery, Princess Elisabeth Children's Hospital, Ghent University Hospital, Ghent, Belgium
a r t i c l e
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Article history: Received 31 October 2016 Accepted 8 November 2016 Key words: Oesophageal atresia Tracheoesophageal fistula Questionnaire Survey Practice
a b s t r a c t Introduction: The surgical management of oesophageal atresia (OA) differs between pediatric surgical teams without consensus. We aimed to describe the current practice of OA treatment in Belgium and Luxembourg and compare this to the literature. Materials and methods: A questionnaire was created and sent to all 18 hospitals (14 pediatric surgical units) performing OA surgery in Belgium and Luxembourg. The results were compared to the literature. Results: Most units treat an average of 2–5 OA + TOF (71%) and ≤1 pure OA (pOA) per year (86%). The preferred surgical approach for OA + TOF is thoracotomy (86%), mostly extra-pleural (75%). Thoracoscopic OA repair is performed in 21%. All centers perform an end-to-end anastomosis (interrupted sutures), and all leave a transanastomotic tube. A chest drain is routinely used in 8 units (57%). In pOA the preferred surgical approach is gastrostomy formation with delayed primary anastomosis (77%). The timing for delayed anastomosis is 2 to 24 months. Intra-operative lengthening is mostly attempted with Foker technique (46%). If oesophageal replacement is needed, gastric interposition is mostly used (75%). A postoperative contrast study is routinely performed in 86% for OA + TOF and in 100% for pOA. Anti-reflux medication is routinely prescribed by all units but one. Conclusion: There are still many differences and controversies in the perioperative management of OA. Part of this is based on habits and is difficult to change without scientific evidence. There is a need for prospective (inter)national registries to further identify the existing differences, leading to a more widely accepted consensus. Level of Evidence: Level III. © 2017 Elsevier Inc. All rights reserved.
Oesophageal atresia (OA) is a rare congenital malformation characterized by an anatomical discontinuity of the oesophagus with or without tracheoesophageal fistula (TOF). The prevalence of OA in reported series varies from 1.2 to 4.5 per 10.000 live births, with a TOF being present in 72% to 94% [1–2]. Many neonates with OA present with other associated anomalies or a genetic syndrome [3]. Their diagnosis is essential to determine the correct medical and surgical management of OA [4]. Since the first attempt to correct OA in 1888, there has been a continuing evolution in surgical techniques, neonatal and anesthetic care, with important impact on survival rates. Current survival rates are around 90%, with almost 100% survival in term neonates without associated anomalies [1,2,4].
⁎ Corresponding author at: Université Libre de Bruxelles (ULB), Department of Paediatric Surgery, Hôpital Universitaire des Enfants Reine Fabiola, Avenue Crocq 15, 1020, Brussels, Belgium. Tel.: +32 496 119 705; fax: +32 2477 34 49. E-mail addresses: [email protected], [email protected] (H. Reusens). 1 Joint first authors. 2 Additional co-authors, names and institutions are cited under Acknowledgements. http://dx.doi.org/10.1016/j.jpedsurg.2016.11.010 0022-3468/© 2017 Elsevier Inc. All rights reserved.
Many steps in the surgical management of OA remain however controversial. Recent (inter)national surveys and cohort studies assessing surgical management strategies of OA have confirmed persisting differences in practice and habits [2,5–7]. The aim of this study was to obtain a complete overview of the actual surgical management of OA amongst all pediatric surgical teams in Belgium and Luxembourg, to describe the differences and variations and compare this to the available literature.
1. Materials and methods Based on the literature, a novel questionnaire on the perioperative management of OA was constructed (Fig. 1). Three independent Belgian pediatric surgeons, board members of the Belgian Association of Pediatric Surgery (BELAPS), reviewed it and could add suggestions. It contained questions concerning prevalence, preoperative investigations, surgical techniques, postoperative management, anti-reflux medication and follow-up. A distinction was made between OA + TOF and pure OA (pOA). The questionnaire was sent to all 18 centers offering pediatric surgical care for OA in Belgium and Luxembourg, to be completed by
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the head of the department or by the team-member with specific interest/ expertise in this topic. Anonymity was guaranteed.
larger ‘Pediatric Surgical Unit’ (PSU) using the same management protocols, 14 PSU were identified for evaluation. 2.2. Cited local OA prevalence and team composition (Table 1)
2. Results 2.1. Survey participation All pediatric surgical teams in Belgium and Luxembourg responded. Since some teams and hospitals replied to be working together as one
Ten PSU (71%) replied to be treating ‘2 to 5 OA + TOF’ per year. Two PSU treat ‘less than 2 OA + TOF’ per year, and two PSU treat ‘between 5 and 10 OA + TOF’ per year. Concerning pOA, 13 of the 14 PSU replied to surgically treat pOA. Most PSU replied to perform ≤1 procedure per year (n = 12, 85%). Two PSU (15%) replied to perform ‘2–4 cases’ per year. In
Fig. 1. Questionnaire used for this survey.
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Fig. 1. (Continue)
Belgium and Luxembourg, there are 30 surgeons treating OA in 14 PSU, with a median of 2 surgeons per PSU (range 1–6).
None of the PSU are performing preoperative contrast studies, CT or MRI. Genetic screening is performed routinely in 4 PSU (29%).
2.3. Preoperative investigations 2.4. Surgical technique in OA + TOF Routine echocardiography is performed preoperatively in all centers for OA + TOF and pOA. A flexible tracheobronchoscopy is routinely performed by 5 PSU (36%) for OA + TOF and by 6 PSU (46%) for pOA. Oesophagoscopy is routinely performed in 1 PSU in case of pOA (7%).
The preferred surgical approach for OA + TOF is thoracotomy (n = 12, 86%) with posterolateral muscle-sparing technique (n = 7, 58%) and extrapleural approach (n = 9, 75%). Thoracoscopy is
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Fig. 1. (Continue)
performed in 3 PSU (21%). In 6 PSU (43%) the azygos vein is preserved during dissection. Intraoperative measurement of the oesophageal gap in case of OA + TOF is routinely performed in 6 PSU (43%): before dissection in 33% (n = 2), before and after dissection in 66% (n = 4). Different methods are used (string, vesselloop, instrument). For pOA, the gap length is routinely measured in all PSU. All PSU perform an end-to-end anastomosis with interrupted sutures. The sutures used are absorbable in 93% (n = 13). Closure of the TOF is mostly performed with absorbable sutures (n = 8, 57%). All
PSU leave an oesophageal transanastomotic tube (TAT). A chest drain is routinely placed in 57% (n = 8). 2.5. Pure and ‘long gap’ oesophageal atresia 2.5.1. Definition of ‘long gap’ The measurement of the gap is expressed in vertebral bodies (VB) in 92% (n = 12) and in centimeters (cm) in 23% (n = 3), one PSU uses both VB and cm. The definition of ‘long gap’ (LG) is controversial. Six PSU (46%) define LG to be a distance of ‘more than 3 VB’. One of these
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Fig. 1. (Continue)
also uses a measurement of ‘more than 3 cm’ as a cut-off value. In two PSU (15%) LG is defined as ‘more than 2 VB’, and one of these equals this with ‘more than 2 cm’ distance. Five PSU (38%) only use ‘impossible primary anastomosis’ to define LG. Overall, ‘impossible primary anastomosis’ is for 8 PSU (62%) an acceptable definition for LG. 2.5.2. Surgical approach Pure OA is treated in 13 of the 14 PSU (93%). The preferred surgical approach is delayed primary anastomosis (DPA) with gastrostomy formation without oesophagostomy in 10 PSU (77%). One PSU (8%) attempts a primary anastomosis without gastrostomy or oesophagostomy. Two PSU (15%) perform DPA with routine gastroand oesophagostomy. The median delay for DPA is 5 months (range 2–24). Four PSU (31%) perform a routine thoracoscopic repair, but most favor a thoracotomy (n = 9, 69%). Preoperative elongation techniques are performed in 6 PSU (46%), mainly by serial bougienage (n = 5, 83%). Intra-operative lengthening is attempted by 7 PSU (54%), mainly by the Foker technique (n = 6, 86%)
or by a spiral myotomy (n = 2, 28%). In one PSU flap oesophagoplasty has been used. There is a full consensus to leave a TAT. A chest drain is routinely placed in 77% (n = 10). If necessary, oesophageal replacement is performed in 61% of PSU (n = 8), with a preference for gastric interposition (n = 6, 75%). In 1 PSU (12%) colonic interposition is performed as a standard technique for all pOA and LG. In another PSU both gastric and colonic interpositions are performed.
2.6. Postoperative management Routine prolonged ventilation is used in 7 PSU (50%) in OA + TOF and in 8 PSU (61%) in pOA, mainly to reduce tension on the anastomosis (57%–62%) or for respiratory reasons (43%–37%). This paralysis is continued for an average of 2.3 days (range 2h-5d). Routine head flexion is used in 1 PSU (8%) in OA + TOF and in 4 PSU (31%) in pOA.
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Table 1 Overview of surveys and large prospective cohort studies on OA. SURVEYS
Number of participants Prevalence OA/TOF: 2–5/year * 5–10/year
PROSPECTIVE COHORTS
Current report
Zani 2014
Shawyer 2014
Lal 2013
Ron 2009 (long gap)
Burge 2013
Sfeir 2013
Pini Prato 2015
14 PSU 30 surgeons
160 surgeons
57 surgeons
170 surgeons
69 surgeons
28 PSU
38 PSU
52 PSU
71% 14%
31% 36%
b5/center 26% 21%
1–3/surgeon 67% 4–7/surgeon 24%
Prevalence pOA: 3–5/surgeon 20% N5/surgeon 7%
Prevalence OA: x x x x
50% N5/center 15%
PSU: Pediatric Surgical Unit; OA/TOF: Oesophageal Atresia with Tracheoesophageal Fistula; pOA: pure Oesophageal Atresia.
Routine contrast study is performed by 86% (n = 12) in OA + TOF and in 100% (n = 13) in pOA at a median of 6 postoperative days (range 3–10). This is reflected in the start of oral feeding after postoperative day (POD) 5 in 12 PSU (85%). In one PSU oral feeding is started without contrast study on POD 1 or 2. Enteral feeding through the TAT is administered in 92% (n = 13), starting on POD 3 to 4 in 57%, and starting before POD 2 in 14%. When pOA, all PSU first perform contrast study before starting oral feeding after POD 5. In these patients, enteral feeding (TAT) is administered in 38% (n = 5) at POD 3–4 and in 46% (n = 6) after POD 5.
2.7. Postoperative anti-reflux medication and follow-up Thirteen PSU (93%) routinely prescribe anti-reflux medication postoperatively. The duration of the medical treatment varies: three months (n = 3, 23%), six months (n = 2, 15%) to 12 months (n = 7, 54%). In one PSU, the duration depends upon findings at routine oesophagogastroduodenoscopy (OGD). Routine postoperative OGD is performed in 3 PSU (21%) for OA/TOF and in 4 PSU (31%) for pOA. The duration of postoperative follow-up varies: ‘less than 1 year’ in one PSU (7%), ‘between 1 and 5 years’ in three PSU (21%), ‘between 5 and 10 years’ in three PSU (21%), ‘between 10 and 16 years’ in two PSU (14%) and ‘more than 16 years’ in five PSU (37%).
3. Discussion This survey is the result of the voluntary joint effort of all pediatric surgical units in Belgium and Luxembourg. It reflects the current perioperative management of OA, based upon a complete (100%) response rate.
3.1. Prevalence/Center volume (Table 1) A bit of caution is needed when interpreting the prevalence and center volume numbers in this survey: all numbers were cited by the centers themselves and not further verified. In larger centers, there may be more than one surgeon performing OA surgery, which may decrease the number of procedures per individual surgeon. In small centers all procedures will possibly be performed by only one surgeon. In this survey, 50% of the teams reported that only a selected number of surgeons perform surgery for OA. Only a few studies on OA repair report on volumeoutcome results. A recent large cohort study in France showed no significant correlation of caseload per center and survival, fistula recurrence, anastomotic stenosis, or oesophageal replacement rates [2]. In contrast, a recent study on thoracoscopic OA repair in Japan showed that all anastomotic leakages occurred in ‘high volume’ centers (N10 OA/year) [7]. The question remains whether concentrating congenital oesophageal pathology in high volume centers or dedicating pediatric surgeons subspecialised in foregut pathology, or a combination of both, will offer a beneficial effect on the postoperative outcomes of OA repair.
3.2. Preoperative investigations (Table 2) Although consensus exists on the routine preoperative use of cardiac ultrasonography in this survey, the use of routine tracheobronchoscopy is still controversial (36–46%), corresponding to the literature (range 21,5–60%) [2,6,8,9]. More than 50% of patients with OA have associated anomalies, more than 20% are born with a VACTERL association or genetic syndrome [1,3,6,10]. Nonetheless, only 4 PSU (30%) in this survey perform a genetic work-up. This number is fairly low, but its interpretation remains difficult as this question wasn't included in other published studies.
Table 2 Overview of results in surveys and large prospective cohort studies on OA. SURVEYS
PROSPECTIVE COHORTS
Current report
Zani 2014
Shawyer 2014
Lal 2013
Burge 2013
Sfeir 2013
Pini Prato 2015
Pre-op echocardiogram Pre-op bronchoscopy
100% 36% OA + TOF 46% pOA
81% 43%
x x
83.2% x
x 21.5%
98.6% 47%
Routine use thoracoscopy
21% OA + 31% pOA 57% OA + 77% pOA 100% 50% OA + 61% pOA 86% OA + 100% pOA 93%
TOF
6%
x
x 60% routine 13% selective 50%
2.5%
x
3%
TOF
69%
70%
83%
53.7%
x
91%
TOF
90% 56%
85% x
84% 25%
98.3% 89,5%
x x
88% x
TOF
72%
70%
85%
36.3%
x
84%
x
86%
76%
51.6
86%
x
Chest drain TAT Prolonged ventilation Contrast study Reflux medication
OA + TOF: oesophageal atresia with tracheoesophageal fistula; pOA: pure oesophageal atresia; TAT: transanastomotic tube; x: no data available.
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3.3. Operative approach
3.4. Postoperative management
3.3.1. Thoracoscopic approach A recent meta-analysis confirmed the effectivity and safety of thoracoscopic OA repair with comparable outcomes to conventional open repair [11]. In our survey, 21% of PSU perform thoracoscopic repair. Although neonatal thoracoscopy becomes increasingly popular [9,12], the amount of centers routinely performing it for OA remains low (Table 2), e.g. only 6% in the EUPSA-BAPS survey [8]. In contrast, the IPEG survey showed thoracoscopic repair in 50% [9], which can be explained by the IPEG members' keen interest in minimally invasive techniques. Other studies mention a lower incidence or do not mention thoracoscopy at all [2,5,6].
3.4.1. Contrast study The use of routine oesophageal contrast studies before the initiation of oral feeding, has since long been debated [18–20]. In Belgium and Luxembourg 86% of PSU does a routine contrast study (100% after pOA repair). In other studies this ranges from 70 to 85% (Table 2). The only exception is the UK, where only 36.3% perform a postoperative contrast study [5]. The authors state that routine contrast study rarely detects a leak and it may delay commencement of feeds and prolong hospital stay.
3.3.2. Surgical technique When attempting a primary oesophageal anastomosis by thoracotomy, the extrapleural approach is the current standard practice in Belgium and Luxembourg (75%). This is comparable to the results in the UK (79%) [5] and Italy (85%) [6]. The type of incision is not fully agreed upon, as approximately 20–25% prefer the axillary incision [6,8,10], above the muscle-sparing posterolateral incision [8]. In this survey, all surgeons agree that the oesophago–oesophageal anastomosis should be performed as an end-to-end anastomosis with interrupted sutures. Most surgeons use absorbable sutures, similar to the IPEG survey [9]. There is also full consensus on leaving a TAT, which is reflected in literature (Table 2). Leaving a chest drain is a topic of ongoing debate [13,14]. A randomized prospective study has shown that a prophylactic extra-pleural chest drain does not decrease early respiratory complications and mortality rates [14]. However, 57% of PSU in the current survey routinely leave a chest tube, which is comparable to other studies (Table 2).
3.3.3. Pure OA The preferred surgical approach in case of pOA is a delayed primary anastomosis with formation of a gastrostomy without oesophagostomy (77%), comparable with 60% of surgeons in the EUPSA-BAPS survey [8] and in agreement with other authors [2,5]. Spitz suggested that “it is generally unproductive to wait longer than 12 weeks in the expectation that the gap will narrow sufficiently to permit a primary anastomosis” [15]. However 23% of PSU wait up to 1 (or 2) years to perform a DPA and two PSU directly opt for oesophageal replacement by colon (at 1 year) or stomach (at 2 months) without attempted elongation or lengthening. In the EUPSA-BAPS survey the delay for DPA ranged between 1 to 12 months (mean 3 months) [8]. In the Italian study the median age of anastomosis was 63 days (range 28–100) [6], which is comparable to the UK (mean 78 days, range 5–135) [5]. Concerning the elongation of the oesophageal ends, there are many different opinions leading to a wide range of surgical habits. Following the ideas of Howard and Foker that “One's own esophagus is best” [12,16], in more than 75% of PSU, techniques are used to attempt delayed primary anastomosis in case of pOA and LGOA: by natural growth, by serial bouginage (39%) with or without gastric bolus feeds, as well as by intraoperative or staged traction if necessary (46%) [5,7,8,15]. Only three PSU (23%) routinely use myotomy or flap reconstruction of the oesophageal ends [5]. When oesophageal replacement is necessary, the majority (70–87%) prefer using the stomach, citing good long-term functional outcome and a satisfied surgeon [2,8,9,12]. This can relate to the ease and reliability of the procedure, as cited by Spitz [8,10,12]. Colonic interposition is only performed by two centers in Belgium and Luxembourg. The use of jejunal interposition in OA patients is rarely first choice for oesophageal replacement [8,9]. A recent meta-analysis failed to show any advantage of one replacement technique over the others [17].
3.4.2. Feeding Although all PSU use a TAT, most PSU (85%) wait with initiating oral feeding until POD 5 or when the contrast oesophagogram shows no leakage. Most PSU will start to administer total parenteral nutrition (TPN) during the first postoperative days. One PSU (7%) starts oral feeding within 24–48 h after surgery without contrast study, a practice also cited by Burge et al. [5], which may shorten the duration of TPN by shortening the times to full enteral and full oral feeding [21]. 3.4.3. Prolonged ventilation Routine prolonged ventilation with paralysis is used by 50% of PSU in this survey, which is comparable with other surveys (Table 2). The median duration is between 2 and 3 days [2,5,8], but may be longer than 7 days in some centers [9]. The main reason for prolonged ventilation is to reduce anastomotic complications by decreasing anastomotic tension [22,23]. Currently, there is little scientific rationale for this technique. The most common indication is actually ‘surgical habit’ or the request by neonatologists, anesthetists or intensivists [5]. 3.4.4. Anti-reflux medication OA is associated with a higher incidence of GORD because of inherent dysmotility of the esophagus and lower oesophageal sphincter [24], or anatomical changes after surgical repair (vagal nerve damage, shortened intra-abdominal esophagus) [25]. GORD may lead to early postoperative complications such as oesophagitis, stricture formation, aspiration pneumonia or failure to thrive [25,26]. In this survey 92% of PSU routinely prescribe anti-reflux medication postoperatively. Comparable to the literature (Table 2), there is however no consensus regarding the use, duration, or type of medication [25]. Some authors recommend anti-reflux medication for at least 6 to 12 months, or even indefinitely, if the patient has symptoms [25,27,28]. 3.4.5. Follow-up In their study comparing 101 adults post OA repair with a control group [29], Rintala et al. described important morbidity in adult OA patients, with long-term problems such as dysphagia, GORD, respiratory symptoms, asthma, infections and also scoliosis and rib cage deformities after open repair. Especially the incidence of GORD in adult survivors of OA is significantly higher than in the general population (33% vs. 10%) [29]. OA survivors may also have a statistical risk of Barrett metaplasia and oesophageal cancer that is (up to 500-fold) greater than that of the general population (although the overall cancer risk seems similar between both groups). These findings indicate the need for a longterm follow-up for all OA patients with an active oesophageal surveillance program. Fifty percent of the PSU in our survey replied to be following the patients for more than ten years, similar to other recent surveys [8], but in need of improvement. 4. Conclusion The results of this survey illustrate that in Belgium and Luxembourg, as in other parts of the world, there is still no general consensus regarding the surgical management of OA.
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(Inter)nationally shared definitions and management guidelines may optimize healthcare by improving patient outcomes and reducing costs. Especially in complex surgical domains, guidelines may improve results, costs and quality of care [9]. In the absence and impossibility of randomized controlled trials for OA, (inter)national multicentric OA registries may provide a more accurate picture of the incidence and outcomes of OA-management. Conflict of interest None. Acknowledgements We sincerely would like to thank all pediatric surgeons in Belgium and Luxembourg and members of the Belgian Association of Pediatric Surgery (BELAPS) who voluntarily cooperated in this international survey. The following pediatric surgeons and BELAPS members, in alphabetical order, are to be acknowledged as co-authors of this paper: Willy Coosemans1, Martine Dassonville2, Antoine De Backer3, Martine Demarche4, Beelke D'hondt5, Marc Dirix6, Paul Leyman7,8, Pierre Lingier9, Paul Philippe10, Anna Poupalou11, Raymond Reding5, Martin Ruppert12, Koenraad Schwagten 7,8, Alexandre Targnion13, Sebastiaan Van Cauwenberge14, Dirk Van de putte15, Erwin Van Der Veken2,16, Erik Van Hoorde17 and Dirk Vervloessem7, 8. 1 University Hospital Gasthuisberg, KUL, Leuven, Belgium; 2 Hôpital Universitaire des Enfants Reine Fabiola, ULB, Brussels, Belgium; 3 Brussels University Hospital, VUB, Brussels, Belgium; 4 CHR de la Citadelle, Liège, Belgium; 5 Cliniques Universitaires St-Luc, UCL, Woluwé, Belgium, 6 CHC Clinique de l'Espérance, Liège, Belgium; 7 AZ St-Augustinus, Antwerp, Belgium; 8 Queen Paola Children's Hospital, ZNA, Antwerp, Belgium; 9 Hôpital Erasme, ULB, Brussels, Belgium; 10 Clinique Pédiatrique, CHL, Luxembourg, Luxembourg; 11 Hôpital Saint-Pierre, ULB, Brussels, Belgium; 12 Antwerp University Hospital, UA, Antwerp, Belgium; 13 CHR de Namur, Namur, Belgium; 14 AZ St-Jan, Bruges, Belgium; 15 Ghent University Hospital, Ghent, Belgium; 16 Hôpital de Jolimont, La Louvière, Belgium; 17 CHU Hôpital Civil Marie Curie, Charleroi, Belgium. References [1] Pedersen RN, Calzolari E, Husby S, et al. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child 2012; 97:227–32. [2] Sfeir R, Bonnard A, Khen-Dunlop N, et al. Esophageal atresia: data from a national cohort. J Pediatr Surg 2013;48:1664–9. [3] Geneviève D, de Pontual L, Amiel J, et al. Genetic factors in isolated and syndromic esophageal atresia. J Pediatr Gastroenterol Nutr 2011;52(Suppl. 1):S6–8. [4] Wang B, Tashiro J, Allan BJ, et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res 2014;190:604–12.
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