|Year : 2022 | Volume
| Issue : 6 | Page : 728-734
Technical innovations to reduce complication rates in esophageal atresia with particular reference to long-term outcomes: A single surgeon's experience of 22 years
Ahmed Morsi, Devesh Misra
Department of Paediatric Surgery, The Royal London Hospital, London, England, UK
|Date of Submission||03-May-2022|
|Date of Decision||12-Jul-2022|
|Date of Acceptance||10-Sep-2022|
|Date of Web Publication||11-Nov-2022|
Department of Paediatric Surgery, The Royal London Hospital, London E1 1BB, England
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Background: Following esophageal atresia/tracheoesophageal fistula (EA/TEF) repair, the standard leak rate reported in the literature is 5%–10%, and stricture rate is 40%–72%. There is a global quest for surgical innovations to drive down these complication rates which can cause considerable morbidity.
Methods: A prospectively maintained database of the senior author's patients who had esophageal atresia repair from 1995 to 2016 was reviewed. Two distinct innovations were implemented: (1) adequate or generous mobilization of the lower esophageal pouch and (2) a 2–5 mm slit in distal esophagus to widen its circumference.
Results: Forty-three patients with EA/TEF were reviewed. Of those, 40 underwent primary repair. The median follow-up was 12.5 years (range 4–26 years). There were no anastomotic leaks and only 8 (20%) patients developed anastomotic strictures requiring dilations (1–5 dilations/patients). One patient (2.5%) had a recurrent fistula. One early mortality was recorded. At the latest follow-up, 35 (87.5%) patients had normal oral feeding, while 1 (2.5%) patient had occasional food sticking episodes. Four syndromic patients (10%) were on jejunal or gastrostomy feeding.
Conclusion: An adequate or generous mobilization of the distal esophageal pouch, together with a 2–5 mm slit in the distal esophagus, achieves a tension-free and wide anastomosis. All anastomoses eventually narrow, sometimes just a little, and starting on a higher scale with a small slit, helps. These seemingly minor innovations, when used together, contributed to a substantially lower complication rate sustained over a 22-year period – no leaks and only 20% stricture rate.
Keywords: Atresia, esophageal, fistula, leak, stricture
|How to cite this article:|
Morsi A, Misra D. Technical innovations to reduce complication rates in esophageal atresia with particular reference to long-term outcomes: A single surgeon's experience of 22 years. J Indian Assoc Pediatr Surg 2022;27:728-34
|How to cite this URL:|
Morsi A, Misra D. Technical innovations to reduce complication rates in esophageal atresia with particular reference to long-term outcomes: A single surgeon's experience of 22 years. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Dec 7];27:728-34. Available from: https://www.jiaps.com/text.asp?2022/27/6/728/360968
| Introduction|| |
Surgery for esophageal atresia and tracheoesophageal fistula (EA/TEF) is technically challenging and can result in complications including anastomotic stricture, leak, dysmotility, recurrent fistula, gastroesophageal reflux disease (GERD), and tracheomalacia., Most international series report a leak rate of 5%–10% and a stricture rate of 40%–72% (mean 50%) as an acceptable norm.,,,, A series published in 2021 reported a stricture rate of 72% in thoracoscopically repaired EA patients.
Esophageal anastomotic stricture is defined as a narrowing at the level of the anastomosis with functional impairment and symptoms and is the most common complication following EA/TEF repair., This is usually confirmed by a contrast study and/or esophagoscopy and can be associated with gastrointestinal or respiratory symptoms.,,
Back in 1995, the senior author decided to challenge certain dogmas and introduced two significant innovations in his practice. The first was ensuring an adequate or sometimes generous mobilization of the distal esophageal pouch and the second was making a tiny slit in the distal esophagus. The purpose of this study was to analyze his experience of 22 years and find out if these innovations had contributed to improved outcomes and lower complication rates, particularly strictures and leaks.
Patients were followed up for 26 years in some cases and underwent manometric and imaging studies, which makes this perhaps the longest follow-up study reported in EA/TEF.
| Methods|| |
Patients who had surgical repair of EA/TEF by the senior author between January 1995 and December 2016 were identified from a prospectively maintained database. Data collected included demographics, clinical and operative details, postoperative contrast study, complications, and follow-up and outcomes. Patients who had end-to-end esophageal anastomosis, whether primary or delayed primary, were the focus of the study.
All patients underwent a conventional open thoracotomy with an extrapleural approach. In all, adequate and sometimes generous mobilization of the distal esophageal pouch was performed, except in rare cases of overlapping esophageal ends where it was deemed unnecessary. The extent of mobilization was as much as was necessary to ensure that both ends lay together comfortably resulting in a tension-free anastomosis (A radical esophageal mobilization down to the diaphragm, as described in long gap EA, was not needed in any patient).
In addition, a small slit of 2–5 mm was made in the distal esophageal end to attain a wider caliber [Figure 1]. Initially, only a tiny slit of 2 mm is made. This is extended if needed, and if feasible – the wider the distal pouch and lesser the tension between the two ends, the longer can the slit be. As per standard international practice, the upper pouch was always mobilized as much as necessary. The esophageal repair was done with full-thickness interrupted sutures of 5/0 or 6/0 polyglactin sutures.
In all patients, a transanastomotic nasogastric tube (TAT) was inserted and tube feeds started after 48 h. Oral feeds were introduced after 7 days and the TAT was removed when it was no longer necessary, usually 2–4 days following oral feeds. Total parenteral nutrition was almost never required.
Ventilation was provided only for those patients where there was a long gap and the anastomosis was under tension. These patients were paralyzed and ventilated for 5 days.
A contrast study was not employed routinely following surgery. It was only done in selected cases where the anastomosis was under tension, or later if patients presented with symptoms of esophageal stenosis. Stricture was defined in this study as any anastomotic narrowing requiring one or more dilation. These patients were put on antacid therapy with ranitidine for a few weeks till their strictures had resolved. Results are presented as mean, median, and range as relevant.
| Results|| |
Over the 22-year period from 1995 to 2016, 43 consecutive patients with EA/TEF were operated by the senior author:
- An extremely sick neonate was born at 24 weeks gestation with a birth weight of 425 g and severe pulmonary hypertension. He underwent an emergency thoracotomy for ligation of TEF. Postoperatively, he died from persistent pulmonary hypertension before definitive EA repair
- Two patients underwent gastric transposition for long gap EA at 3–5 months
- The remaining 40 patients had primary or delayed primary end-to-end esophageal anastomosis. This group is the focus of the current study.
Gestational age ranged from 24 to 40 weeks (median = 37 weeks). These neonates had birth weights ranging from 425 to 3940 g (median weight = 2420 g). [Table 1] summarizes patients from b to c (In 2017, the senior author went off on calls and therefore stopped doing EA/TEF surgery).
|Table 1: Patients who had definitive esophageal atresia/tracheoesophageal fistula repair|
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The median follow-up duration for the 40 patients was 12.5 years (range 4–26 years). A postoperative contrast esophagogram was performed in only seven of 40 patients on day 7. There was no clinical or radiological evidence of leaks in any of our patients. One patient developed a postoperative chylothorax which was proven on biochemical and cytological analysis – he resolved with 7 days of fasting and parenteral nutrition. There were no other cases of pleural effusion.
Eight patients (20%) developed an esophageal anastomotic stricture and needed dilations as follows:
- From 1995 to 2003, four patients underwent dilations using gum elastic bougies (range 1–4 dilations/patient)
- From 2004, we switched over to fluoroscopic balloon dilations. There were four patients who had 1–5 dilatations each.
These eight patients all had obstructive symptoms and underwent a contrast swallow before esophageal dilatation which confirmed anastomotic narrowing together with hold up of contrast. All strictures resolved with dilations alone and did not require further intervention, i.e. the strictures were mild. One (2.5%) patient was diagnosed with a recurrent TEF at the age of 1 year following a prone tube esophagogram done in the context of recurrent chest infections and choking episodes. She had a successful redo thoracotomy for the repair of recurrent TEF.
The stricture rate in the first half of the study (1995–2005) and in the second half (2006–2016), was identical.
The following associated anomalies were found: VACTERL association (n = 12), CHARGE syndrome (n = 1), cardiac (n = 5), renal (n = 4), CNS (n = 3), anorectal malformations (n = 5 – 3 high ARM, 1 anterior anus, and 1 cloaca), and duodenal atresia (n = 1). Pyloromyotomy for infantile pyloric stenosis was performed on one patient. Alopecia areata was noted in a patient who later developed chronic myeloid leukemia at age 9 years; he survived after successful therapy. Suxamethonium anaphylaxis was encountered in one patient at the time of surgery.
At the last follow-up, 35/40 (87.5%) patients had normal oral feeding, with 1 (2.5%) patient having occasional self-resolving food sticking episodes. Four syndromic patients (10%) have continued jejunal/gastrostomy feeding, in three patients partially and in one patient exclusively (the latter also has a tracheostomy). On long-term follow-up, esophageal dysmotility was documented in 13 (32.5%) patients. Of these, two patients >20 years of age were investigated by adult gastroenterologists and were found to have complete esophageal aperistalsis.
Nine patients (22.2%) aged 5–26 years have occasionally used proton pump inhibitors to treat reflux-related symptoms. Of these, two patients (5%) had fundoplication for significant GERD resistant to medical treatment and one patient (2.5%) with neurological impairment had hiatus hernia repair. Recurrent mild chest infections were reported in 11 (27.5%) patients in early childhood and four had bronchial asthma needing steroid therapy. Two aortopexies (5%) were performed for significant tracheomalacia.
There were five deaths in our series. In addition to the extremely premature baby born at 24 weeks gestation mentioned earlier, four late mortalities were identified, the causes of death being nongastrointestinal [Table 2].
|Table 2: Mortalities in 43 patients with esophageal atresia/tracheoesophageal fistula repair|
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To compare with a control group, we looked at the outcomes of other consultants in the same department. From 2003 to 2016, 94 EA/TEF patients had primary repair: 22 by the senior author implementing the aforementioned innovations and 72 by other consultants with varying techniques.
Stricture rate in the senior author's group for that period was 22.7% and there were no leaks. For the other consultants, 35/72 (48.6%) patients had anastomotic strictures and 5/72 (6.9%) patients had anastomotic leaks, one of whom (1.3%) required redo thoracotomy. A Chi-square test of independence showed a significant difference in stricture rates between the senior author and the rest of the department (P = 0.031).
| Discussion|| |
Since the first description of successful repair of EA in 1943, survival has improved with advancing anesthetic and surgical techniques.,,, Current survival rates in newborns with TEF/EA range from 91% to 97% and may reach 100% in infants born full term without associated significant cardiac or chromosomal anomalies.,,
Gastrointestinal complications are common and may result from strictures, gastroesophageal reflux or dysmotility. Anastomotic stricture remains the most common complication following surgical repair of TEF/EA and is reported in 40%–79% of the patients with an average of about 50%.,, Risk factors for anastomotic strictures include prematurity, low birth weight, VACTERL association, and the presence of a long gap with consequent anastomotic tension. In addition, refractory strictures are seen following anastomotic leaks and in the early postoperative strictures. GERD is also a proposed contributor for strictures.,
Strictures can cause significant morbidity. There have been case reports of perforations following esophageal dilations. Partly, the problem results from the fact that at the initial thoracotomy, the distal esophageal pouch can be very narrow. In 1995, the senior author made a conscious decision to routinely slit the lower esophageal pouch for a distance varying from 2 to 5 mm. It should be remembered that the circumference of the distal esophagus can sometimes be only 8 mm, and in such patients, even a tiny 2 mm slit increases the circumference by 4 mm [Figure 1]b, resulting in a 1/3 increase in the circumference of the organ!
This may be a tiny modification, but at the end of the day, a stricture rate of only 20% over 22 years speaks for itself. This compares with series being published even today which show a stricture rate of 40%–72% in EA/TEF patients.,
Avoiding excessive dissection and mobilization of the distal esophageal pouch has been an unspoken taboo over years for fear of jeopardizing the “segmental” blood supply with potentially increased risks for anastomotic stricture, leak, and dysmotility. However, the vascular anatomy of the esophagus has been extensively studied and there is no full agreement among the anatomists on the sources of the esophageal blood supply.,
In 1992, Schärli reported five long gap EA patients who had their distal esophagus mobilized by ligation and division of the left gastric artery with division of the lesser curve of the stomach. He reported no cases of esophageal necrosis, potentiating the assumption that the short gastric and gastroepiploic arteries provide additional blood supply to the distal esophagus.
In 1995, the senior author decided to challenge this dogma and decided to do, in all patients, an adequate, and sometimes generous mobilization of the distal esophageal pouch, as much as was necessary to get the two ends to lie together comfortably. The belief was that such anastomoses are less likely to leak, and also less likely to stenose. This has been gratifyingly demonstrated over the 22 years with no patient developing an anastomotic leak (never reported before), including the few who had a long gap and actually needed a 5-day paralysis and postoperative ventilation. In literature, a small study of five patients has been reported of long gap EA who underwent radical mobilization of the lower esophageal pouch down to the diaphragm with no side effects.
A review of global literature of complications following EA surgery is presented in [Table 3].
|Table 3: International review of anastomotic strictures and leaks following esophageal atresia/tracheoesophageal fistula repair|
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Our study, being a single-author series, has understandably a smaller number of patients.
There is a series of 95 patients from a tertiary level referral center which only looked at type C patients. Their stricture rate was 53.6%, and for thoracoscopic cases, it was 72%. The BAPS CASS study was done nationwide across the United Kingdom and Ireland but had a limitation of being focused only on outcomes up to 1 year of life, not later. The multicenter study from the United States of 396 patients showed a high leak rate of 23% and even had a dehiscence rate of 2%. This high rate of leaks, as also the mention of dehiscence reported in the two series from Canada and the USA is disturbing and emphasizes the fact that standards vary widely.,, There should be a move to limit EA/TEF repair to centers of excellence where there would be an adequate number of cases per year to allow the surgeons to enhance their skills, as also practice innovations such as ours with careful audits every few years to validate their success rates.
Indeed, in the UK, the national GIRFT study is a step in the right direction, with a national discussion ongoing about the need to centralize EA surgery to fewer centers. The number of hours of training in pediatric surgery in the UK has declined from 30,000 in 1995 to an average of about 8000 today, mainly due to the European working time directive. This has been reflected in the level of confidence and experience of a few newly appointed consultants, and it has become the policy in some centers that EA surgery should be done by two consultants jointly, till the surgeons gain experience and become independent operators. Certainly, there is anecdotal evidence of a nationwide increase in the incidence of major complications over the past few years (inability to find the fistula, bronchial or vascular injuries, etc.) and measures such as these serve to support the younger consultants.
In our series, although we had no patients with documented anastomotic leaks, we are conscious that we did not perform routine contrast studies in all patients which could have picked up tiny, localized leaks. However, the significance of these tiny leaks is debatable. From 1995 onward, the senior author has only used contrast studies postoperatively where the anastomosis was under tension, or later if the patients presented with symptoms of dysphagia or vomiting.
In literature, “anastomotic leak” is a very broad term that continues to be applied to a wide range of radiological and clinical events.,, We believe there should be a unified consensus when it comes to describing anastomotic leaks. Description would ideally include how the anastomotic leak is diagnosed (radiological or clinical) and whether the leak is managed conservatively or required surgical intervention. For radiological leaks, the indication for contrast studies should be mentioned, whether done routinely or indicated for clinical deterioration. Furthermore, surgically addressed anastomotic leaks should be quantified as partial disruption or complete dehiscence of the suture line.
The low rate of antireflux procedures in our study reflects our own high threshold for fundoplication. In EA/TEF patients, there is a higher risk of redo fundoplication due to esophageal dysmotility, and the wrap can increase lower esophageal resistance and further complicate swallowing.
Recent recommendations have been made to standardize the management of EA/TEF patients. Furthermore, attempts to achieve better outcomes have been put to test, e.g., a no Tube, no prosthesis protocol was prospectively applied across multiple USA centers. However, there was no reduction in leak or stricture rates between pre- and postprotocol patient cohorts.
Two of our adult survivors had complete esophageal aperistalsis, which initially surprised us. However, this is now being reported as a recognizable motility disorder in long-term EA/TEF survivors, together with pressurization and distal peristalsis patterns as detected on high-resolution esophageal manometry. Both patients are currently under follow-up by adult gastroenterologists with no further procedures so far. Furthermore, EA/TEF patients carry higher risks of developing Barrett's esophagus and eosinophilic esophagitis., Some of our older patients have been on proton pump inhibitors intermittently or have had some trouble with chest infections and asthma. Interestingly, we also found significant chest problems in our achalasia patients followed into adult life.
On a somber note, with the oldest survivors in their seventies now, cases of esophageal malignancies are beginning to emerge. Therefore, individualized transition processes and long-term surveillance for EA/TEF patients are essential to improve the health consequences of this congenital but lifelong problem.
In a changing world, where some surgeons are doing EA/TEF repair thoracoscopically, our series serves as a standard to compare with. A zero leak rate in a long series covering over two decades has never been reported before. Centers should constantly strive to audit their results and reduce the incidence of surgical complications following this exquisitely difficult operation.
This study represents a small but significant paradigm shift in establishing lower complication rates following EA/TEF repair (no leaks, and only a 20% stricture rate). This was achieved by an adequate or sometimes generous mobilization of the lower esophageal pouch. We believe that esophageal ends that lie comfortably together are less likely to leak and less likely to stenose.
Furthermore, a tiny 2–5 mm slit in the distal esophagus increases the circumference of this rather narrow organ and contributes to a lower stricture rate. All anastomoses eventually narrow, sometimes just a little, and starting on a higher scale, as it were, helps.
All strictures were mild and needed a few dilations only.
This series is the first in the world to use these two innovations together, and we feel that they contributed to the substantially lower complication rates. Our long follow-up, ranging up to 26 years, allowed us to document unusual findings of esophageal aperistalsis or dysmotility in a few adult survivors.
Although prospectively collected, we are aware of the chances of bias in reporting what is essentially a single surgeon series. While we have focused on two innovations, we are aware that surgical techniques alter subtly over a long period, and some of the refinements that have crept in are difficult to describe fully and may reflect the skill or experience of the senior surgeon. The latter may partly explain the better outcomes in this series.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3]