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Surgical management of oesophageal atresia
Paediatric Respiratory Reviews 19 (2016) 10–15
Contents lists available at ScienceDirect
Paediatric Respiratory Reviews
Mini-symposium: Oesophageal Atresia and Tracheo-oesophageal Fistula
Surgical management of oesophageal atresia Warwick J. Teague 1,2,3, Jonathan Karpelowsky 4,5,* 1
Academic Paediatric Surgeon, Department of Paediatric Surgery, The Royal Children’s Hospital, Melbourne, VIC, Australia Clinical Associate Professor, Department of Paediatrics, The Royal Children’s Hospital, Melbourne, VIC, Australia 3 Honorary Fellow, Surgical Research Group, Murdoch Children’s, Melbourne, VIC, Australia 4 Paediatric Surgeon, Department of Paediatric Surgery, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead 2145, Sydney, NSW, Australia 5 Senior Lecturer, Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Sydney, NSW, Australia 2
EDUCATIONAL AIMS The reader will come to appreciate how: 1. 2. 3. 4. 5.
To To To To To
understand the rationale for preoperative work up in oesophageal atresia describe the surgical correction of oesophageal atresia review the role of thoracoscopic surgery in oesophageal atresia describe surgical options to correct long-gap oesophageal atresia review early post-operative complications.
A R T I C L E I N F O
S U M M A R Y
Keywords: Oesophageal atresia Long Gap oesophageal atresia Tracheo-oesophageal fistula
There have been major advances in the surgery for oesophageal atresia (OA) and tracheo-oesophageal fistula(TOF) with survival now exceeding 90%. The standard open approach to OA and distal TOF has been well described and essentially unchanged for the last 60 years. Improved survival in recent decades is most attributable to advances in neonatal anaesthesia and perioperative care. Recent surgical advances include the use of thoracoscopic surgery for the repair of OA/TOF and in some centres isolated OA, thereby minimising the long term musculo-skeletal morbidity associated with open surgery. The introduction of growth induction by external traction (Foker procedure) for the treatment of long-gap OA has provided an important tool enabling increased preservation of the native oesophagus. Despite this, long-gap OA still poses a number of challenges, and oesophageal replacement still may be required in some cases. ß 2016 Elsevier Ltd. All rights reserved.
INTRODUCTION The first successful repair of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) was performed in 1941: Cameron Haight ligated the TOF prior to an end-to-end oesophageal anastomosis through a left extrapleural approach [1]. In the subsequent 75 years the overall survival has improved to exceed 90%, with mortality now usually associated with prematurity and/ or cardiac comorbidity [2,3]. Despite improved survival, OA still
* Corresponding author. Department of Paediatric Surgery, The Children’s Hospital at Westmead, University of Sydney, Locked Bag 4001, Westmead 2145, Sydney, NSW, Australia. Tel.: +61 2 9845 3341; fax: +61 2 9845 3180. E-mail address: [email protected] (J. Karpelowsky). http://dx.doi.org/10.1016/j.prrv.2016.04.003 1526-0542/ß 2016 Elsevier Ltd. All rights reserved.
presents unique surgical challenges, many of which are discussed here. This review focuses on the surgical management of OA and any associated TOF, highlighting the preoperative investigations, timing of surgery, surgical approaches, surgical complications and challenges of ‘long-gap’ OA. PREOPERATIVE ASSESSMENT The two primary goals of preoperative assessment in the patient with a clinical diagnosis of OA are: 1. Confirmation of the diagnosis; 2. Identification of associated anomalies with immediate management implications for the planned oesophageal atresia surgery.
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Table 1 Frequency of associated anomalies in OA (Data extracted from Burge et al. 2013 [4]) Associated Anomaly
All OA
OA with distal TOF
Other OA subtypes
Any Vertebral Anorectal Cardiac Renal Limb VACTERL Chromosomal
52% 7% 11% 34% 9% 11% 13% 3%
54% 6% 6% 36% 8% 14% 12% -
44% 16% 40% 28% 16% 3% 24% -
As more than half of OA patients have an associated anomaly (Table 1), [4] additional systems screening for anomalies is ordinarily deferred until after primary OA surgery. Exceptions to this are outlined briefly below. The clinical diagnosis of OA is made when a 10 French (Fr) tube arrests in the upper oesophagus approximately 10-12 cm from the lips. Tubes of narrower calibre may give the false impression of tube passage by coiling in the dilated upper oesophageal pouch, or rarely traversing the trachea and TOF to enter the stomach [5,6]. A diagnosis of OA is then confirmed with a plain chest x-ray to show the arrested 10 Fr tube within the relative lucency of the dilated upper oesophageal pouch. An accompanying distal TOF is indicated by the presence of gastrointestinal gas. Particular attention is paid to marked stomach dilatation, which may reflect preferential ventilation of the stomach via the TOF, or concomitant duodenal atresia with a ‘double bubble’. In either setting, emergency distal TOF ligation is indicated to prevent the rare but morbid complication of gastric perforation [7]. Conversely, a ‘gasless abdomen’ on preoperative x-ray raises the possibility of either pure OA or OA with a proximal TOF, and should prompt further investigation as detailed below. The chest x-ray should also be assessed for evidence of early complications (e.g. pulmonary aspiration, intubation) or associated anomalies (e.g. abnormalities of the cardiac silhouette, vertebrae and ribs). A preoperative echocardiogram is undertaken to define any associated major congenital heart disease (CHD), particularly ductdependent lesions which may necessitate particular anaesthetic management or prior cardiac surgery. In addition, an echocardiogram may demonstrate vascular anomalies relevant to operative decision-making, most notably a right-sided aortic arch (RAA) which is present in approximately 4% of OA cases [8]. The importance of routine preoperative echocardiography is highlighted by expert commentators, [5,6] whilst others present data to moderate this stance, [9,10] showing preoperative echocardiography findings of CHD and/or RAA seldom alter the operative plan, including the choice of right vs left thoracotomy in cases with a known RAA [8,10]. The role of routine preoperative laryngotracheobronchoscopy (LTB) in OA patients remains a matter of debate, [11] with only 43-60% of contemporary paediatric surgeons routinely using preoperative LTB in this setting [12,13]. Advocates cite preoperative LTB findings which may impact management in 21-45% of OA patients, most notably unusual fistula position (Figure 1) and tracheobronchial tree anomalies, and less commonly laryngeal clefts or subglottic stenosis [14–16]. The presence of significant tracheomalacia may alert clinicians to the need for non-invasive support following extubation. The benefit of preoperative LTB is maximal in newborns with suspected pure OA due to a ‘gasless abdomen’ on x-ray. In this OA subgroup, LTB reveals a proximal TOF in 20-33% with resultant change in management [14–17]. An upper pouch oesophagogram may augment LTB to identify a
Figure 1. Bronchoscopy of a distal tracheo-oesophageal fistula.
‘near-missed’ proximal TOF, [18] but is highly dependent on local radiology expertise. Preoperative endoscopic intubation of the TOF with the aim facilitating surgical repair, Fogarty balloon TOF occlusion to aid ventilation and selective trans-tracheal gastric drainage have been described [11,15,16,19]. Routine screening investigations for other associated anomalies would include renal ultrasound, spinal ultrasound and sacral x-ray. These are ordinarily not performed preoperatively, except a renal ultrasound in the newborn who has not voided (OA is uncommonly associated with renal agenesis), [6] or urgent genetic testing where an undiagnosed lethal syndrome is suspected, e.g. Trisomy 13 (Patau) or 18 (Edwards). OPEN SURGERY With rare exceptions, OA surgery is not an emergency and can be deferred whilst preoperative investigations are obtained and an appropriately skilled anaesthetic and surgical team assembled. Indications for emergency OA surgery are limited to a markedly dilated stomach at risk of perforation, or the premature infant with evolving Respiratory Distress Syndrome (RDS) in whom preferential TOF ventilation is a significant contributor to a deteriorating ventilatory status. Two recent studies report increased complications in OA patients undergoing surgery within 24 hours of birth or after-hours [20,21]. Selection biases aside, these studies highlight the benefits of in-hours OA surgery, which may be safely deferred beyond 24 hours of life. The goals and key steps of the open approach have remained largely unchanged over decades, and are summarised below. Specific goals and operative strategies for other OA variants, e.g. long-gap OA, are described elsewhere in this review. Key operative goals 1. TOF ligation to prevent further soiling of the tracheobronchial tree with stomach contents and restore the ventilation dynamics of the intact trachea. 2. Restoration of oesophageal continuity, which can be deferred if the child’s status is poor at completion of TOF ligation.
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Key operative steps 1. Right posterolateral thoracotomy, with attention to a musclesparing technique to reduce the musculoskeletal sequelae of thoracotomy in infancy [22]. 2. Extrapleural approach to the posteromedial mediastinum, to expose the azygos vein (typically, but not universally, ligated and divided in continuity) and the TOF. TOF dissection (Figure 2) is minimised with an aim of preserving its neurovascular supply, and proceeds to expose the junction with the trachea in readiness for TOF ligation. 3. TOF ligation is undertaken, with either an absorbable or nonabsorbable suture. Prior to division of the ‘fistula’, it is important ensure neither a major bronchus nor aorta have been mistakenly dissected as iatrogenic injuries to each have occurred. Some advocate peroperative tracheoscopy to optimise TOF dissection and ligation, [19] but this technique is not yet been widely adopted. 4. Upper oesophageal pouch dissection (Figure 3) optimally commences prior to TOF ligation. Downward pressure by the
Figure 4. Completed thoracoscopic anastomosis.
anaesthetist on the 10Fr oesophageal tube indicates the approximate position of the upper pouch, allowing unexpected positions to be taken into account at TOF ligation. Following TOF ligation, upper pouch dissection proceeds until adequate length is obtained or the pouch is maximally dissected. Sharp and careful dissection of the plane between the trachea and pouch is the key to avoiding tracheal injury during this step. Finally, the upper pouch is incised in readiness for anastomosis. 5. Oesophageal anastomosis (Figure 4) with fine interrupted sutures begins with the back wall, following which a transanastomotic nasogastric tube is passed prior to completion of the anastomosis of the front wall. THORACOSCOPIC SURGERY
Figure 2. Dissection of trachea-oesophageal fistula.
Figure 3. Dissection of Upper blind ending pouch in oesophageal atresia.
The benefits of minimally invasive surgery over thoracotomy for reducing pain, scars and long term musculoskeletal deformities including scoliosis have been well documented [23–25]. The first thoracoscopic repair of an isolated OA was undertaken in 1999, [26] and OA with distal TOF in 2000 [27]. Since then, a number of larger multi-institutional series have validated the thoracoscopic technique to be equivalent to open surgery [28–30]. The patient is positioned semi prone with the right side elevated by 30 degrees. Standard endotracheal intubation is used with no need for endobronchial isolation. Three 3 mm ports are used, although some surgeons use a 5 mm port to facilitate use of an endoclip. A pneumothorax of 3-5 mmHg facilitates lung collapse and visualisation, and a fourth port may be used should lung retraction become necessary. Port placement is crucial due to the limited working space afforded by the neonatal thorax. As in open surgery, the first step is to use the azygos vein and vagus nerve to identify the TOF. The azygos vein is often divided (energy device, clips or ties), but may be preserved. The TOF is dissected cranially to its junction with the membranous trachea, prior to being ligated flush with the trachea using either a suture or endoclip. Attention is then turned to the upper pouch, which is identified and dissected with the aid of gentle pressure on the 10 Fr tube in the upper pouch. The anastomosis is technically challenging, and performed using interrupted sutures secured by intracorporeal ties. Once complete, a chest tube may be left through one of the port site incisions. Technical benefits of the thoracoscopic approach include improved visualisation of the entire hemithorax and excellent magnification [31]. Approaching the fistula perpendicularly allows
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dissection in situ with localisation of the point of entry into the membranous part of the trachea. Dissection of the upper pouch is also improved, with straightforward ability to extend high up into the neck if required. Despite the stated benefits and comparability to the open approach, the highly skilled nature of thoracoscopic OA surgery has limited its wider adoption [24]. A recent survey of paediatric surgeons noted open thoracotomy was the preferred approach by 94% of surgeons, [12] albeit this number is reduced to only 50% when surveying a cohort of surgeons with an interest in minimally invasive surgery [13]. Concerns have been raised regarding the physiological stresses experienced by the neonate during thoracoscopic repair, especially hypercapnoea resulting in acidosis and cerebral hypoperfusion [32]. Recent infrared spectroscopy data however, goes some way to addressing these concerns by showing hypercapnoea during thoracoscopic OA surgery is not associated with cerebral hypoxia [33].
When oesophageal preservation is not possible, several replacement methods are available [36]. Gastric ‘pull up’ involves mobilising the entire stomach on the right gastric and right gastroepiploic arteries into the posterior mediastinum and suturing the fundus to the proximal oesophagus [49]. Conversely, a gastric ‘tube’ involves tubularisation of the greater curve of the stomach leaving a smaller but functional stomach. The gastric tube is then translocated into the thorax on the right gastro-epiploic vessels [41]. Finally, colonic [50] and jejunal [51] interposition involve isolating a segment of intestine with its mesenteric pedicle, and ‘interposing’ this conduit between the proximal oesophagus and stomach. Despite each of these replacement options providing a reliable conduit, the surgery is associated with a high incidence of complications (10-45%) including mortality in 4-5% [41,52]. No one conduit has proven to be superior, rather familiarity with the techniques and local expertise determine success.
LONG-GAP OESOPHAGEAL ATRESIA
The three early postoperative complications of OA surgery with greatest relevance to the patient’s medium and long-term outcome are: anastomotic leak (3-20%), anastomotic stricture (39-57%) and recurrent TOF (3-7%); cited incidences coming from recent British and German multicentre prospective cohort studies [53,54].
The surgical management of long-gap OA still remains a significant challenge [34–36]. Currently, there is no consensus on the definition of long-gap OA. Many discussions have focussed only on pure OA, [37] although a recent meta-analysis confirms many long-gap OA occur together with a distal TOF [38]. Greater consensus exists to support prioritising the retention of the native oesophagus, rather than proceeding primarily with oesophageal replacement. Replacement is however, an important option for a select cohort of OA patients, in whom ongoing attempts to retain the native oesophagus are deemed futile and likely detrimental to the child’s wellbeing [39,40]. Various methods have been developed to overcome the technical difficulties of long-gap OA. Methods to preserve the oesophagus include delayed primary anastomosis and lengthening procedures such as circular myotomy, oesophageal flap, Foker procedure (traction suture oesophageal lengthening) and Kimura technique (multistage extra-thoracic oesophageal elongation). Alternatively, oesophageal replacement may be performed with conduits, including gastric tube, gastric transposition, small bowel or colonic interposition [41]. Delayed primary anastomosis involves creating a gastrostomy to facilitate feeding at or soon after birth. At this time, a ‘gap study’ may be performed to assess the length of the gap, e.g. dilator is passed via the stomach into the distal oesophageal pouch and with a large firm tube in the proximal oesophagus a gap is measured using fluoroscopy. The gap length is typically expressed as ‘number of vertebral bodies’. Many surgeons consider a gap exceeding two vertebral bodies to be difficult to anastomose primarily, i.e. ‘longgap OA’. In this instance, the child is fed via gastrostomy and the upper pouch managed by continuous aspiration of saliva. When performed, the anastomosis can be achieved by open or thoracoscopic approach, with or without adjuncts to maximise length, e.g. upper pouch flaps or myotomies [31,42]. Since first described in in 1997, [43] the Foker and other traction-based procedures have gained popularity [44–48]. This staged procedure involves primary mobilization of the oesophageal ends prior to placement of and externalisation of traction sutures. Tension on the externalised sutures is then gradually increased over days to encourage elongation of the upper and lower pouches to narrow the ‘gap’. Whether traction induces true growth in the oesophageal ends, or only stretch, remains controversial [23]. Once sufficient length has been achieved a primary oesophageal anastomosis is undertaken. The placement of traction sutures and subsequent anastomosis can be undertaken by an open or thoracoscopic approach [46].
EARLY POSTOPERATIVE COMPLICATIONS
Anastomotic Leak Anastomotic leaks occur in 3–20% of OA patients [53–56]. Reported incidences may reflect local utilisation of ‘routine’ contrast studies to scrutinise the anastomosis, e.g. on the 5th postoperative day, rather than technical skill. The key risk factor for leakage is anastomotic tension [57]. Major leaks occur in 3-5% and manifest early (<48 hours) with acute deterioration, including tension pneumothorax and sepsis. Emergency chest tube decompression is effective to control the associated pleural soiling, and in many cases no other operative intervention is required. Re-do thoracotomy, with or without oesophagostomy, is an uncommon event, reserved for complete anastomotic disruption, persistent soiling or inability to control pneumothoraces [5,55,56]. Minor leaks are more common, contained and typically noted on ‘routine’ contrast studies in otherwise asymptomatic children. Minor leaks seldom require specific management, and can be expected to close spontaneously following a brief deferral of oral feeds [55]. The use of glycopyrolate, which reduces saliva production, has been suggested as an adjunct to encourage closure [58]. The key long-term sequelae of anastomotic leakage include an increased risk of troublesome stricture formation or recurrent TOF [55,56]. Anastomotic Stricture Anastomotic stricture is common, occurring in approximately one third of OA patients. The key risk factors for stricture formation are anastomotic tension, gastro-oesophageal reflux and previous anastomotic leak [55,57,59–61]. Serial dilatation is a safe and effective treatment, be that using balloon or bougie techniques [57,60–62]. Both approaches are associated with a low risk of perforation (<2% per dilatation episode), albeit that the vast majority of perforations are managed non-operatively [59,60,62]. Recurrent TOF Recurrent fistula formation is an uncommon but morbid complication, which may present with recurrent chest infections and acute life-threatening events and prove difficult to diagnose [63]. Treatment may be by an open or endoscopic approach, the
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latter including thermal ablation using laser or diathermy followed by occlusion with fibrin or Cyanoacrylate glue [64,65]. The role of endoscopic management remains a matter of controversy. A recent systematic review found open surgery to be superior to endoscopy with lower morbidity, fewer treatment episodes and greater success [66]. However, the less invasive nature of endoscopic management makes this a suitable option for a well-selected patient. Vocal cord paresis Vocal cord paresis (VCP) is reported in approximately 3-4% of patients post OA surgery [67,68]. The presumed aetiology is intraoperative injury to the recurrent laryngeal nerve. This notwithstanding, congenital VCP has also been reported in OA patients, highlighting a benefit of preoperative LTB. Whilst VCP cases manifest overtly with early stridor or failed extubation, others present diagnostic difficulties [68,69]. Unilateral VCP is managed expectantly and may resolve spontaneously, whereas bilateral VCP requires operative management with tracheostomy [68]. LATE POSTOPERATIVE COMPLICATIONS The interplay of gastro-oesophageal reflux and/or tracheomalacia with OA is a cause of significant morbidity, including acute life-threatening events. Medical and surgical management each have a role to mitigate such complications [70,71]. These and other long-term aerodigestive complications and their management are discussed elsewhere in this mini-symposium, and are the subject of a recent meta-analysis [72]. FUTURE RESEARCH DIRECTIONS Future research in surgical therapy for oesophageal atresia needs to focus on several key areas: 1. There is a need for standardisation of the definition of ‘long-gap OA’ and taxonomy of the procedures used to address this particular OA subtype. This advance would facilitate comparative and collaborative research investigating surgical management of this challenging OA subtype. 2. There should be evaluation of the long-term complications of open compared with thoracoscopic surgery, to evaluate whether the stated benefits of a technically more difficult but less invasive technique are warranted. 3. There is a need for investment in basic research to overcome barriers currently preventing creation of a biological conduit suitable for use in oesophageal replacement.
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health insurance database covering a population of 8 million. Dis Esophagus 2015. Zhao R, Li K, Shen C, Zheng S. The outcome of conservative treatment for anastomotic leakage after surgical repair of esophageal atresia. J Pediatr Surg 2011;46:2274–8. Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ. Anastomotic leakage following surgery for esophageal atresia. J Pediatr Surg 1992;27:29–32. McKinnon LJ, Kosloske AM. Prediction and prevention of anastomotic complications of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1990;25:778–81. Mathur S, Vasudevan SA, Patterson DM, Hassan SF, Kim ES. Novel use of glycopyrrolate (Robinul) in the treatment of anastomotic leak after repair of esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2011; 46:e29–32. Serhal L, Gottrand F, Sfeir R, Guimber D, Devos P, Bonnevalle M, Storme L, Turck D, Michaud L. Anastomotic stricture after surgical repair of esophageal atresia: frequency, risk factors, and efficacy of esophageal bougie dilatations. J Pediatr Surg 2010;45:1459–62. Said M, Mekki M, Golli M, Memmi F, Hafsa C, Braham R, Belguith M, Letaief M, Gahbiche M, Nouri A, et al. Balloon dilatation of anastomotic strictures secondary to surgical repair of oesophageal atresia. Br J Radiol 2003;76:26–31. Baird R, Laberge JM, Levesque D. Anastomotic stricture after esophageal atresia repair: a critical review of recent literature. Eur J Pediatr Surg 2013;23:204–13. Levesque D, Baird R, Laberge JM. Refractory strictures post-esophageal atresia repair: what are the alternatives? Dis Esophagus 2013;26:382–7. Coran AG. Diagnosis and surgical management of recurrent tracheoesophageal fistulas. Dis Esophagus 2013;26:380–1. Lal DR, Oldham KT. Recurrent tracheoesophageal fistula. Eur J Pediatr Surg 2013;23:214–8. Meier JD, Sulman CG, Almond PS, Holinger LD. Endoscopic management of recurrent congenital tracheoesophageal fistula: a review of techniques and results. Int J Pediatr Otorhinolaryngol 2007;71:691–7. Aworanti O, Awadalla S. Management of recurrent tracheoesophageal fistulas: a systematic review. Eur J Pediatr Surg 2014;24:365–75. Morini F, Iacobelli BD, Crocoli A, Bottero S, Trozzi M, Conforti A, Bagolan P. Symptomatic vocal cord paresis/paralysis in infants operated on for esophageal atresia and/or tracheo-esophageal fistula. J Pediatr 2011;158:973–6. Oestreicher-Kedem Y, DeRowe A, Nagar H, Fishman G, Ben-Ari J. Vocal fold paralysis in infants with tracheoesophageal fistula. Ann Otol Rhinol Laryngol 2008;117:896–901. Mortellaro VE, Pettiford JN, St Peter SD, Fraser JD, Ho B, Wei J. Incidence, diagnosis, and outcomes of vocal fold immobility after esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) repair. Eur J Pediatr Surg 2011;21: 386–8. Shawyer AC, D’Souza J, Pemberton J, Flageole H. The management of postoperative reflux in congenital esophageal atresia-tracheoesophageal fistula: a systematic review. Pediatr Surg Int 2014;30:987–96. Jennings RW, Hamilton TE, Smithers CJ, Ngerncham M, Feins N, Foker JE. Surgical approaches to aortopexy for severe tracheomalacia. J Pediatr Surg 2014;49:66–70. discussion 70–61. Connor MJ, Springford LR, Kapetanakis VV, Giuliani S. Esophageal atresia and transitional care–step 1: a systematic review and meta-analysis of the literature to define the prevalence of chronic long-term problems. Am J Surg 2015;209:747–59.
Contents lists available at ScienceDirect
Paediatric Respiratory Reviews
Mini-symposium: Oesophageal Atresia and Tracheo-oesophageal Fistula
Surgical management of oesophageal atresia Warwick J. Teague 1,2,3, Jonathan Karpelowsky 4,5,* 1
Academic Paediatric Surgeon, Department of Paediatric Surgery, The Royal Children’s Hospital, Melbourne, VIC, Australia Clinical Associate Professor, Department of Paediatrics, The Royal Children’s Hospital, Melbourne, VIC, Australia 3 Honorary Fellow, Surgical Research Group, Murdoch Children’s, Melbourne, VIC, Australia 4 Paediatric Surgeon, Department of Paediatric Surgery, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead 2145, Sydney, NSW, Australia 5 Senior Lecturer, Discipline of Paediatrics & Child Health, Sydney Medical School, University of Sydney, Sydney, NSW, Australia 2
EDUCATIONAL AIMS The reader will come to appreciate how: 1. 2. 3. 4. 5.
To To To To To
understand the rationale for preoperative work up in oesophageal atresia describe the surgical correction of oesophageal atresia review the role of thoracoscopic surgery in oesophageal atresia describe surgical options to correct long-gap oesophageal atresia review early post-operative complications.
A R T I C L E I N F O
S U M M A R Y
Keywords: Oesophageal atresia Long Gap oesophageal atresia Tracheo-oesophageal fistula
There have been major advances in the surgery for oesophageal atresia (OA) and tracheo-oesophageal fistula(TOF) with survival now exceeding 90%. The standard open approach to OA and distal TOF has been well described and essentially unchanged for the last 60 years. Improved survival in recent decades is most attributable to advances in neonatal anaesthesia and perioperative care. Recent surgical advances include the use of thoracoscopic surgery for the repair of OA/TOF and in some centres isolated OA, thereby minimising the long term musculo-skeletal morbidity associated with open surgery. The introduction of growth induction by external traction (Foker procedure) for the treatment of long-gap OA has provided an important tool enabling increased preservation of the native oesophagus. Despite this, long-gap OA still poses a number of challenges, and oesophageal replacement still may be required in some cases. ß 2016 Elsevier Ltd. All rights reserved.
INTRODUCTION The first successful repair of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) was performed in 1941: Cameron Haight ligated the TOF prior to an end-to-end oesophageal anastomosis through a left extrapleural approach [1]. In the subsequent 75 years the overall survival has improved to exceed 90%, with mortality now usually associated with prematurity and/ or cardiac comorbidity [2,3]. Despite improved survival, OA still
* Corresponding author. Department of Paediatric Surgery, The Children’s Hospital at Westmead, University of Sydney, Locked Bag 4001, Westmead 2145, Sydney, NSW, Australia. Tel.: +61 2 9845 3341; fax: +61 2 9845 3180. E-mail address: [email protected] (J. Karpelowsky). http://dx.doi.org/10.1016/j.prrv.2016.04.003 1526-0542/ß 2016 Elsevier Ltd. All rights reserved.
presents unique surgical challenges, many of which are discussed here. This review focuses on the surgical management of OA and any associated TOF, highlighting the preoperative investigations, timing of surgery, surgical approaches, surgical complications and challenges of ‘long-gap’ OA. PREOPERATIVE ASSESSMENT The two primary goals of preoperative assessment in the patient with a clinical diagnosis of OA are: 1. Confirmation of the diagnosis; 2. Identification of associated anomalies with immediate management implications for the planned oesophageal atresia surgery.
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Table 1 Frequency of associated anomalies in OA (Data extracted from Burge et al. 2013 [4]) Associated Anomaly
All OA
OA with distal TOF
Other OA subtypes
Any Vertebral Anorectal Cardiac Renal Limb VACTERL Chromosomal
52% 7% 11% 34% 9% 11% 13% 3%
54% 6% 6% 36% 8% 14% 12% -
44% 16% 40% 28% 16% 3% 24% -
As more than half of OA patients have an associated anomaly (Table 1), [4] additional systems screening for anomalies is ordinarily deferred until after primary OA surgery. Exceptions to this are outlined briefly below. The clinical diagnosis of OA is made when a 10 French (Fr) tube arrests in the upper oesophagus approximately 10-12 cm from the lips. Tubes of narrower calibre may give the false impression of tube passage by coiling in the dilated upper oesophageal pouch, or rarely traversing the trachea and TOF to enter the stomach [5,6]. A diagnosis of OA is then confirmed with a plain chest x-ray to show the arrested 10 Fr tube within the relative lucency of the dilated upper oesophageal pouch. An accompanying distal TOF is indicated by the presence of gastrointestinal gas. Particular attention is paid to marked stomach dilatation, which may reflect preferential ventilation of the stomach via the TOF, or concomitant duodenal atresia with a ‘double bubble’. In either setting, emergency distal TOF ligation is indicated to prevent the rare but morbid complication of gastric perforation [7]. Conversely, a ‘gasless abdomen’ on preoperative x-ray raises the possibility of either pure OA or OA with a proximal TOF, and should prompt further investigation as detailed below. The chest x-ray should also be assessed for evidence of early complications (e.g. pulmonary aspiration, intubation) or associated anomalies (e.g. abnormalities of the cardiac silhouette, vertebrae and ribs). A preoperative echocardiogram is undertaken to define any associated major congenital heart disease (CHD), particularly ductdependent lesions which may necessitate particular anaesthetic management or prior cardiac surgery. In addition, an echocardiogram may demonstrate vascular anomalies relevant to operative decision-making, most notably a right-sided aortic arch (RAA) which is present in approximately 4% of OA cases [8]. The importance of routine preoperative echocardiography is highlighted by expert commentators, [5,6] whilst others present data to moderate this stance, [9,10] showing preoperative echocardiography findings of CHD and/or RAA seldom alter the operative plan, including the choice of right vs left thoracotomy in cases with a known RAA [8,10]. The role of routine preoperative laryngotracheobronchoscopy (LTB) in OA patients remains a matter of debate, [11] with only 43-60% of contemporary paediatric surgeons routinely using preoperative LTB in this setting [12,13]. Advocates cite preoperative LTB findings which may impact management in 21-45% of OA patients, most notably unusual fistula position (Figure 1) and tracheobronchial tree anomalies, and less commonly laryngeal clefts or subglottic stenosis [14–16]. The presence of significant tracheomalacia may alert clinicians to the need for non-invasive support following extubation. The benefit of preoperative LTB is maximal in newborns with suspected pure OA due to a ‘gasless abdomen’ on x-ray. In this OA subgroup, LTB reveals a proximal TOF in 20-33% with resultant change in management [14–17]. An upper pouch oesophagogram may augment LTB to identify a
Figure 1. Bronchoscopy of a distal tracheo-oesophageal fistula.
‘near-missed’ proximal TOF, [18] but is highly dependent on local radiology expertise. Preoperative endoscopic intubation of the TOF with the aim facilitating surgical repair, Fogarty balloon TOF occlusion to aid ventilation and selective trans-tracheal gastric drainage have been described [11,15,16,19]. Routine screening investigations for other associated anomalies would include renal ultrasound, spinal ultrasound and sacral x-ray. These are ordinarily not performed preoperatively, except a renal ultrasound in the newborn who has not voided (OA is uncommonly associated with renal agenesis), [6] or urgent genetic testing where an undiagnosed lethal syndrome is suspected, e.g. Trisomy 13 (Patau) or 18 (Edwards). OPEN SURGERY With rare exceptions, OA surgery is not an emergency and can be deferred whilst preoperative investigations are obtained and an appropriately skilled anaesthetic and surgical team assembled. Indications for emergency OA surgery are limited to a markedly dilated stomach at risk of perforation, or the premature infant with evolving Respiratory Distress Syndrome (RDS) in whom preferential TOF ventilation is a significant contributor to a deteriorating ventilatory status. Two recent studies report increased complications in OA patients undergoing surgery within 24 hours of birth or after-hours [20,21]. Selection biases aside, these studies highlight the benefits of in-hours OA surgery, which may be safely deferred beyond 24 hours of life. The goals and key steps of the open approach have remained largely unchanged over decades, and are summarised below. Specific goals and operative strategies for other OA variants, e.g. long-gap OA, are described elsewhere in this review. Key operative goals 1. TOF ligation to prevent further soiling of the tracheobronchial tree with stomach contents and restore the ventilation dynamics of the intact trachea. 2. Restoration of oesophageal continuity, which can be deferred if the child’s status is poor at completion of TOF ligation.
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Key operative steps 1. Right posterolateral thoracotomy, with attention to a musclesparing technique to reduce the musculoskeletal sequelae of thoracotomy in infancy [22]. 2. Extrapleural approach to the posteromedial mediastinum, to expose the azygos vein (typically, but not universally, ligated and divided in continuity) and the TOF. TOF dissection (Figure 2) is minimised with an aim of preserving its neurovascular supply, and proceeds to expose the junction with the trachea in readiness for TOF ligation. 3. TOF ligation is undertaken, with either an absorbable or nonabsorbable suture. Prior to division of the ‘fistula’, it is important ensure neither a major bronchus nor aorta have been mistakenly dissected as iatrogenic injuries to each have occurred. Some advocate peroperative tracheoscopy to optimise TOF dissection and ligation, [19] but this technique is not yet been widely adopted. 4. Upper oesophageal pouch dissection (Figure 3) optimally commences prior to TOF ligation. Downward pressure by the
Figure 4. Completed thoracoscopic anastomosis.
anaesthetist on the 10Fr oesophageal tube indicates the approximate position of the upper pouch, allowing unexpected positions to be taken into account at TOF ligation. Following TOF ligation, upper pouch dissection proceeds until adequate length is obtained or the pouch is maximally dissected. Sharp and careful dissection of the plane between the trachea and pouch is the key to avoiding tracheal injury during this step. Finally, the upper pouch is incised in readiness for anastomosis. 5. Oesophageal anastomosis (Figure 4) with fine interrupted sutures begins with the back wall, following which a transanastomotic nasogastric tube is passed prior to completion of the anastomosis of the front wall. THORACOSCOPIC SURGERY
Figure 2. Dissection of trachea-oesophageal fistula.
Figure 3. Dissection of Upper blind ending pouch in oesophageal atresia.
The benefits of minimally invasive surgery over thoracotomy for reducing pain, scars and long term musculoskeletal deformities including scoliosis have been well documented [23–25]. The first thoracoscopic repair of an isolated OA was undertaken in 1999, [26] and OA with distal TOF in 2000 [27]. Since then, a number of larger multi-institutional series have validated the thoracoscopic technique to be equivalent to open surgery [28–30]. The patient is positioned semi prone with the right side elevated by 30 degrees. Standard endotracheal intubation is used with no need for endobronchial isolation. Three 3 mm ports are used, although some surgeons use a 5 mm port to facilitate use of an endoclip. A pneumothorax of 3-5 mmHg facilitates lung collapse and visualisation, and a fourth port may be used should lung retraction become necessary. Port placement is crucial due to the limited working space afforded by the neonatal thorax. As in open surgery, the first step is to use the azygos vein and vagus nerve to identify the TOF. The azygos vein is often divided (energy device, clips or ties), but may be preserved. The TOF is dissected cranially to its junction with the membranous trachea, prior to being ligated flush with the trachea using either a suture or endoclip. Attention is then turned to the upper pouch, which is identified and dissected with the aid of gentle pressure on the 10 Fr tube in the upper pouch. The anastomosis is technically challenging, and performed using interrupted sutures secured by intracorporeal ties. Once complete, a chest tube may be left through one of the port site incisions. Technical benefits of the thoracoscopic approach include improved visualisation of the entire hemithorax and excellent magnification [31]. Approaching the fistula perpendicularly allows
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dissection in situ with localisation of the point of entry into the membranous part of the trachea. Dissection of the upper pouch is also improved, with straightforward ability to extend high up into the neck if required. Despite the stated benefits and comparability to the open approach, the highly skilled nature of thoracoscopic OA surgery has limited its wider adoption [24]. A recent survey of paediatric surgeons noted open thoracotomy was the preferred approach by 94% of surgeons, [12] albeit this number is reduced to only 50% when surveying a cohort of surgeons with an interest in minimally invasive surgery [13]. Concerns have been raised regarding the physiological stresses experienced by the neonate during thoracoscopic repair, especially hypercapnoea resulting in acidosis and cerebral hypoperfusion [32]. Recent infrared spectroscopy data however, goes some way to addressing these concerns by showing hypercapnoea during thoracoscopic OA surgery is not associated with cerebral hypoxia [33].
When oesophageal preservation is not possible, several replacement methods are available [36]. Gastric ‘pull up’ involves mobilising the entire stomach on the right gastric and right gastroepiploic arteries into the posterior mediastinum and suturing the fundus to the proximal oesophagus [49]. Conversely, a gastric ‘tube’ involves tubularisation of the greater curve of the stomach leaving a smaller but functional stomach. The gastric tube is then translocated into the thorax on the right gastro-epiploic vessels [41]. Finally, colonic [50] and jejunal [51] interposition involve isolating a segment of intestine with its mesenteric pedicle, and ‘interposing’ this conduit between the proximal oesophagus and stomach. Despite each of these replacement options providing a reliable conduit, the surgery is associated with a high incidence of complications (10-45%) including mortality in 4-5% [41,52]. No one conduit has proven to be superior, rather familiarity with the techniques and local expertise determine success.
LONG-GAP OESOPHAGEAL ATRESIA
The three early postoperative complications of OA surgery with greatest relevance to the patient’s medium and long-term outcome are: anastomotic leak (3-20%), anastomotic stricture (39-57%) and recurrent TOF (3-7%); cited incidences coming from recent British and German multicentre prospective cohort studies [53,54].
The surgical management of long-gap OA still remains a significant challenge [34–36]. Currently, there is no consensus on the definition of long-gap OA. Many discussions have focussed only on pure OA, [37] although a recent meta-analysis confirms many long-gap OA occur together with a distal TOF [38]. Greater consensus exists to support prioritising the retention of the native oesophagus, rather than proceeding primarily with oesophageal replacement. Replacement is however, an important option for a select cohort of OA patients, in whom ongoing attempts to retain the native oesophagus are deemed futile and likely detrimental to the child’s wellbeing [39,40]. Various methods have been developed to overcome the technical difficulties of long-gap OA. Methods to preserve the oesophagus include delayed primary anastomosis and lengthening procedures such as circular myotomy, oesophageal flap, Foker procedure (traction suture oesophageal lengthening) and Kimura technique (multistage extra-thoracic oesophageal elongation). Alternatively, oesophageal replacement may be performed with conduits, including gastric tube, gastric transposition, small bowel or colonic interposition [41]. Delayed primary anastomosis involves creating a gastrostomy to facilitate feeding at or soon after birth. At this time, a ‘gap study’ may be performed to assess the length of the gap, e.g. dilator is passed via the stomach into the distal oesophageal pouch and with a large firm tube in the proximal oesophagus a gap is measured using fluoroscopy. The gap length is typically expressed as ‘number of vertebral bodies’. Many surgeons consider a gap exceeding two vertebral bodies to be difficult to anastomose primarily, i.e. ‘longgap OA’. In this instance, the child is fed via gastrostomy and the upper pouch managed by continuous aspiration of saliva. When performed, the anastomosis can be achieved by open or thoracoscopic approach, with or without adjuncts to maximise length, e.g. upper pouch flaps or myotomies [31,42]. Since first described in in 1997, [43] the Foker and other traction-based procedures have gained popularity [44–48]. This staged procedure involves primary mobilization of the oesophageal ends prior to placement of and externalisation of traction sutures. Tension on the externalised sutures is then gradually increased over days to encourage elongation of the upper and lower pouches to narrow the ‘gap’. Whether traction induces true growth in the oesophageal ends, or only stretch, remains controversial [23]. Once sufficient length has been achieved a primary oesophageal anastomosis is undertaken. The placement of traction sutures and subsequent anastomosis can be undertaken by an open or thoracoscopic approach [46].
EARLY POSTOPERATIVE COMPLICATIONS
Anastomotic Leak Anastomotic leaks occur in 3–20% of OA patients [53–56]. Reported incidences may reflect local utilisation of ‘routine’ contrast studies to scrutinise the anastomosis, e.g. on the 5th postoperative day, rather than technical skill. The key risk factor for leakage is anastomotic tension [57]. Major leaks occur in 3-5% and manifest early (<48 hours) with acute deterioration, including tension pneumothorax and sepsis. Emergency chest tube decompression is effective to control the associated pleural soiling, and in many cases no other operative intervention is required. Re-do thoracotomy, with or without oesophagostomy, is an uncommon event, reserved for complete anastomotic disruption, persistent soiling or inability to control pneumothoraces [5,55,56]. Minor leaks are more common, contained and typically noted on ‘routine’ contrast studies in otherwise asymptomatic children. Minor leaks seldom require specific management, and can be expected to close spontaneously following a brief deferral of oral feeds [55]. The use of glycopyrolate, which reduces saliva production, has been suggested as an adjunct to encourage closure [58]. The key long-term sequelae of anastomotic leakage include an increased risk of troublesome stricture formation or recurrent TOF [55,56]. Anastomotic Stricture Anastomotic stricture is common, occurring in approximately one third of OA patients. The key risk factors for stricture formation are anastomotic tension, gastro-oesophageal reflux and previous anastomotic leak [55,57,59–61]. Serial dilatation is a safe and effective treatment, be that using balloon or bougie techniques [57,60–62]. Both approaches are associated with a low risk of perforation (<2% per dilatation episode), albeit that the vast majority of perforations are managed non-operatively [59,60,62]. Recurrent TOF Recurrent fistula formation is an uncommon but morbid complication, which may present with recurrent chest infections and acute life-threatening events and prove difficult to diagnose [63]. Treatment may be by an open or endoscopic approach, the
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latter including thermal ablation using laser or diathermy followed by occlusion with fibrin or Cyanoacrylate glue [64,65]. The role of endoscopic management remains a matter of controversy. A recent systematic review found open surgery to be superior to endoscopy with lower morbidity, fewer treatment episodes and greater success [66]. However, the less invasive nature of endoscopic management makes this a suitable option for a well-selected patient. Vocal cord paresis Vocal cord paresis (VCP) is reported in approximately 3-4% of patients post OA surgery [67,68]. The presumed aetiology is intraoperative injury to the recurrent laryngeal nerve. This notwithstanding, congenital VCP has also been reported in OA patients, highlighting a benefit of preoperative LTB. Whilst VCP cases manifest overtly with early stridor or failed extubation, others present diagnostic difficulties [68,69]. Unilateral VCP is managed expectantly and may resolve spontaneously, whereas bilateral VCP requires operative management with tracheostomy [68]. LATE POSTOPERATIVE COMPLICATIONS The interplay of gastro-oesophageal reflux and/or tracheomalacia with OA is a cause of significant morbidity, including acute life-threatening events. Medical and surgical management each have a role to mitigate such complications [70,71]. These and other long-term aerodigestive complications and their management are discussed elsewhere in this mini-symposium, and are the subject of a recent meta-analysis [72]. FUTURE RESEARCH DIRECTIONS Future research in surgical therapy for oesophageal atresia needs to focus on several key areas: 1. There is a need for standardisation of the definition of ‘long-gap OA’ and taxonomy of the procedures used to address this particular OA subtype. This advance would facilitate comparative and collaborative research investigating surgical management of this challenging OA subtype. 2. There should be evaluation of the long-term complications of open compared with thoracoscopic surgery, to evaluate whether the stated benefits of a technically more difficult but less invasive technique are warranted. 3. There is a need for investment in basic research to overcome barriers currently preventing creation of a biological conduit suitable for use in oesophageal replacement.
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