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 Table of Contents  
Year : 2020  |  Volume : 7  |  Issue : 2  |  Page : 42-49

Quagmire of esophageal replacement in infants and children

1 Department of Paediatric Surgery, Government Medical College, Super Specialty Hospital, Nagpur, India
2 Department of Pediatrics, BYL Nair Hospital, Mumbai, Maharashtra, India

Date of Submission17-May-2020
Date of Decision31-Jul-2020
Date of Acceptance23-Oct-2020
Date of Web Publication30-Dec-2020

Correspondence Address:
Dr. Rajendra Govind Saoji
5th Floor, Midas Heights, Ramdaspeth, Nagpur - 440 010, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJAMR.IJAMR_50_20

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A normally functioning esophagus is a specialized functioning organ, and it functions in concert with other derivatives of foregut origin. Unlike adults, esophageal replacement (OR) in infants and children is uniformly done for benign conditions. The complexity of esophageal substitution techniques and its sequelae are not experienced only during childhood, but they spill over into adult life as well making life-long commitment and follow-up necessary. Although multiple technical approaches and their modifications are being practiced for OR over the past century, there is no clear consensus regarding the best one. In recent years, realizing that patient's own esophagus suits him/her best, esophageal lengthening and tissue engineering techniques are challenging the replacement techniques. There are no randomized controlled pediatric studies to compare different types of OR.

Keywords: Caustics, esophageal atresia, esophageal lengthening, esophageal replacement, pediatric esophagus

How to cite this article:
Saoji RG, Saoji AR. Quagmire of esophageal replacement in infants and children. Int J Adv Med Health Res 2020;7:42-9

How to cite this URL:
Saoji RG, Saoji AR. Quagmire of esophageal replacement in infants and children. Int J Adv Med Health Res [serial online] 2020 [cited 2023 Feb 8];7:42-9. Available from: https://www.ijamhrjournal.org/text.asp?2020/7/2/42/305454

  Introduction Top

Restoring continuity between two ends of the esophagus, absent or damaged, is an uphill task. Multiple techniques of esophageal replacement (OR) have evolved over several decades using native tissues – bowel (jejunum or colon), stomach (gastric pull-up [GPU]) and gastric tubes (reversed gastric tube [RGT] or isoperistaltic gastric tube [IGT]). In adults, the most common reason for OR is malignancy, whereas in children, indications are uniformly benign [Table 1]. Thus, normal life expectancy, growth and development, swallowing and nutrition, pulmonary function are important issues. All esophageal grafts are known for variable spectrum of early and late morbidities. Hence, preservation of native esophagus is the best strategy, and corollary to this thinking is delayed primary repair, esophageal lengthening techniques, and regenerative medicine are challenging OR. However, OR would be inevitable in certain situations therefore, pediatric surgeons must remain familiar with this complex procedure and its perioperative care. The aim of this brief yet comprehensive review article is to make the reader aware of current popular esophageal conduits, their complications, sequelae, and an overview of other options available and the future. A detailed search of data on commonly used esophageal conduits, their merits and demerits and their published results in infants and children done from databases-PubMed (NLM), Medline (Ovid) and Cochrane Library (Wiley) was performed for the current review. Additional references were also retrieved by “snowballing” methodology. Author's experience of 103 cases of transhiatal GPU through posterior mediastinum with two other major series, has been included [Table 2].
Table 1: Indications for oesophageal replacement

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Table 2: Esophageal substitution – Transhiatal gastric pull-up with conduit placed in posterior mediastinum: Comparative data of 3 series with 100 or more cases each

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  Evolution of Esophageal Reconstruction Top

The first esophageal reconstruction was reported in 1877 by Czerny.[1],[2] Subsequently, for long gap esophageal atresia (LGOA), William Ladd and Gross performed skin tube OR in stages for children.[3] However, due to nonsatisfactory function, esthetics and potential risk of malignancy soon it became obsolete. Later, a variety of esophageal substitutes were tried, but the quest for an ideal conduit is still an unfinished task.

Jejunum, as a conduit, was first used by Roux in 1907.[4],[5] However, precarious vasculature makes it technically demanding to harvest pedicled or free grafts. Hence, only some centers have reported good outcomes in children. Romanian surgeon Gavriliu is credited for pioneering RGT as a conduit in adults.[6] Although publications and usage of the gastric tube[7],[8],[9],[10],[11] and its modifications[12],[13] remained limited due to high anastomotic leak rate and stricture, it is a good alternative to bridge the gap between esophageal ends. In 1921, Lundblad performed the first successful colonic interposition in a toddler with caustic stricture who succumbed to a car accident 3.5 decades later. Sandblom first reported colon substitute in OA. Colon replacement then was popularized by David Waterston and others.[14],[15],[16],[17] It remains the most commonly used conduit, but due to technical challenges and high complication rates need for another conduit was felt.[18],[19],[20],[21] Stomach with its robust blood supply, simplicity, and do-ability at all hands is currently enjoying popularity as a conduit of choice. Kummel in 1922 first published GPU but replacement of the entire esophagus was done by Sweet in 1948 at Massachusetts General Hospital, USA.[22],[23] However, the resurgence of GPU was done in 1970 by Spitz and team at Great Ormond Street hospital due to dismal outcomes of colonic grafts.[24] Synthetic tubular prosthesis for OR was attempted but never gained popularity.[25],[26],[27],[28] Except the recent introduction of the Minimal Invasive Surgery (MIS) approach, nothing new has been added in the armamentarium of OR in the past several decades. The features of an ideal esophageal substitute are summarized in [Table 3].
Table 3: Expected “ideal” esophageal substitute

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  Principles of Esophageal Reconstruction Top

Preoperative evaluation

Other than routine workup for major reconstructive surgery, three[37] specific useful investigations before OR adds very valuable [38] information.

  1. Contrast study for morphological[39] details of the lower esophageal stump, stomach and colon.[40]
  2. Bronchoscopic evaluation of airway under dynamic and relaxant anesthesia for Laryngo-Tracheomalacia, cartilage abnormality, upper pouch fistula, granuloma, laryngotracheosophageal cleft, etc.[43]
  3. Contrast enhanced computed tomography of mediastinum for[44] vascular abnormalities, mucocele/abscess especially after perforation of oesophagus.

Choice of conduit and surgical technique

All types of OR share three phases of dissection: intra-abdominal, mediastinal, and cervical phase. Common surgical steps to all replacement procedures are – careful selection and harvesting of conduit, resection of the lower esophageal stump or strictured esophagus, and bridging the gap between two esophageal ends. In the absence of randomized controlled trials, conduit selection depends on the familiarity, experience, and expertise of a surgeon. Supraumbilical vertical or transverse laparotomy incision is used to get an access for the intra-abdominal phase.

Currently, MIS approaches viz. Transhiatal laparoscopic or Thoracoscopy + trans-hiatal laparoscopy or Hybrid approach (thoracoscopy + open abdominal transhiatal ) are challenging conventional exclusive open surgical techniques. Thoracoscopy adds to safety by under vision,precise and complete mobilization of esophagus whenever severe peri-esophageal fibrosis is expected, for example, long corrosive stricture, failed primary repair of tracheoesophageal fistula or previous conduit etc. MIS approach is expected to reduce surgical stress and provide a clear magnified vision of mediastinum hence appears attractive. However, laparoscopy requires longer operation time and change of position if thoracoscopy is needed, loss of tactile feel of the mediastinal pathway, technically more demanding, high cost, etc. Thus, as of now, clearly more validation is needed.[29],[30]

In isolated OA, the distal esophageal stump is usually discarded as it remains small and underdeveloped on most of occasions. In the neonatal period, distal stump may be exteriorized as abdominal esophageal feeding stoma to keep the stomach virgin for subsequent OR.[31] Various esophageal substitutes have their advantages, challenges, and specific complications [Table 4]. Irrespective of the replacement technique, the most common morbidity is anastomotic leakage and stricture and more so when gastric tubes and bowels were used as a conduit. Strictures, stump esophagitis, and ulcerations of the small or large bowel conduits are all consequences of the acid reflux in the conduit. This mandates lifelong monitoring for metaplasia-dysplasia-adenocarcinoma.[32]
Table 4: Current oesophageal substitution procedures

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  Intrathoracic versus Cervical Anastomosis Top

To achieve anastomosis in the neck there are three major routes – posterior mediastinal, trans-pleural, and retrosternal.[33] Relative advantages and disadvantages of the different routes are outlined in [Table 5]. The posterior mediastinal route being the shortest, straight and the bed of a native esophagus makes it an ideal bed for the conduit. Thus, if postoperative anastomotic dilatation is needed, it can be done with relative ease as compared to retro-sternal route having inherently associated angulations at cervical and xiphoid levels. Esophageal and conduit anastomosis can either be performed through left or right thoracotomy. However, it is prudent to avoid intrathoracic anastomosis due to the potential risk of anastomotic leak and mediastinitis. Hence, primary cervical anastomosis is preferable. The thoracic inlet can act as constriction to the passage of the esophageal graft. In such situation, the thoracic inlet can be widened surgically by either resecting the left half of the manubrium or sternoclavicular joint and sternal head of the left clavicle. However, diligent and patiently done dissection mostly obviates the need for these maneuvers.
Table 5: Routes of esophageal conduit

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  Factors Predicting Outcome of Oesophageal Replacement Top

Preoperative failure of sham feeding can lead to swallowing difficulties and food aversion despite satisfactory replacement procedure. Dysphagia among these children can be due to anastomotic stricture/stenosis, significant acid reflux, anti-peristaltic conduit, redundancy in graft, dysmotility, or combination of one or more factors. Thus, it is essential to monitor nutrition, growth, and development after OR, particularly for oesophageal atresia as they are known to fall below 15th centiles for weight in postoperative period. Pulmonary functions need careful assessment as mediastinal crowding due to conduit, especially after GPU, may compress lung and reduce lung capacity. Furthermore, these children may develop recurrent chest infections related to aspirations.[34]

Timing of OR varies according to several factors – experience of a surgical team, setting in which care is provided, associated anomalies, cardio-pulmonary status, weight of the baby, etc. Ideally, it should be performed as a nonurgent and well-planned procedure. Though primary GPU is possible in the neonatal period for LGOA, it requires very daunting postoperative care.[35] Therefore, it is prudent to delay the procedure until the infant is thriving and weighs 5 kg and is preferably 3 months of age, all cardio-pulmonary issues are well settled to minimize postoperative complications.[36] This interval also provides an opportunity to explore possibility to retain native esophagus. In all cases, adequate mechanical preparation of the colon is essential because the colon may be required as a conduit if another organ (i.e., stomach and small intestine) proves to be unsuitable intraoperatively. In view of likely complications; chronic inflammation, retrosternal pain, anemia, metaplastic or dysplastic change (Barrett's esophagus), mucocele and empyema; resection of the damaged esophagus is the best strategy.[37],[38],[39],[40]

  Techniques of Various Oesophageal Conduits Top

Commonly used OR conduits include gastric interposition, gastric tube interposition, jejunal interposition, and colonic interposition.

Gastric interposition or transposition/gastric pull up

The stomach is endowed with robust submucosal arterial and venous plexus and therefore, can sustain extensive mobilization on pedicles of greater and lesser curvature arcades [Figure 1]. Gastrostomy stoma is taken down by meticulous separation of adhesions from the liver and anterior abdominal wall. In the case of OA, lower esophageal stump can be delivered without much difficulty by trans-Hiatal posterior mediastinal dissection with due care. The gastroepiploic arcade and the right gastric vessels are preserved. To achieve more length, kocharization may be added. Pyloroplasty or pyloromyotomy is advocated to provide better drainage as vagotomy is inevitable. Proximal esophagus or the esophagostomy is mobilized through transverse neck incision, and posterosuperior mediastinum is entered to create a tunnel with great caution to avoid injury to the recurrent laryngeal nerve, posterior tracheal wall, thoracic duct, and pleura. Thoracotomy or thoracoscopic [Figure 2] approach may be needed to complete the safe resection of the esophagus in case of extensive perioesophageal fibrosis or adhesions from previous surgery or caustic injuries. A transhiatal posterior mediastinal tunnel is completed wide enough to facilitate smooth gliding of stomach up to be delivered in the neck for cervical esophago-gastric anastomosis without any compression, especially at diaphragmatic hiatus and at thoracic inlet level. Particular attention must be paid not to twist the stomach during the pull-up maneuver. Gastroesophageal anastomosis is finally performed in the neck using the apex of the fundus. The trans-anastomotic nasogastric tube is kept for decompression of conduit. Lastly, a feeding jejunostomy is constructed as a safety valve.
Figure 1: Gastric pull up: Open approach. (a) Harvested stomach on right gastric and gastroepiploic vessels. (b) Highest fundic point for anastomosis with oesophageal stump in the neck. (c) Mobilised oesophageal stump + fundus of stomach delivered in the neck. (d) Two layered oesophgo.gastric anastomosis fixed to prevertebral fascia and sternocleidomastoid muscle

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Figure 2: Gastric pull up: MIS approach. (a) Preoperative contrast study showing long caustic oesophageal stricture. (b) Thoracoscopic mobilisation of oesophagus in dissection modified prone position. (c) Laparoscopic approach for abdominal and mediastinal dissection through hiatus. (d) Resectedscarred oesophagus. (e) Pulled up stomach well placed between mediastinal pleural leaves

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Gastric tube interposition

The gastric tube can be created from the greater curvature in a reversed (anteperistaltic) [Figure 3] or isoperistaltic fashion. For RGT, the gastrocolic omentum is divided at a safe distance from greater curvature to preserve gastro-epiploic arcade and lower esophageal stump is resected. RGT is based on the left gastroepiploic pedicle. The greater curvature of the stomach is divided 5 cm proximal to the pylorus. A tube is then constructed from the greater curvature around an 18–24 Fr catheter using stapled or hand-sewn technique. Similarly, an IGT is formed based on the right gastroepiploic artery. It can be done with the preservation of the lower native esophagus and the esophagogastric junction. The esophageal hiatus is enlarged, and the gastric tube is brought into the chest, preferably through the posterior mediastinum. Primary or secondary (depending upon vascularity status of conduit) anastomosis in the neck with the proximal esophagus is performed in a single layer. A trans-anastomotic nasogastric tube is kept and a feeding gastrostomy is also performed. In adults, a comparative study of over 200 patients concluded that GPU has more advantages over the gastric tube.[41]
Figure 3: Reversed gastric tube. (a) Well preserved gastro.epiploic arcade and left gastroepiploic based pedicle. (b) Preparation of RGT on number 24 red rubber tube. (c) Hand sewn reversed gastric tube of adequate length. (d) Preparation of oesophago.gastric tube anastomosis in the neck. (e) Preoperative contrast study showing long caustic oesophageal stricture. (f) Postoperative well placed nonredundant reversed gastric tube 5 years after surgery

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Jejunal interposition

Pedicled or a free graft of jejunum is utilized making to construct esophageal conduit. The preparation of jejunum can be done early at the time of the initial gastrostomy or at a later laparotomy by ligating the vessel in continuity at the base of the jejunal loop to promote hypertrophy of the vascular arcade and to improve venous drainage after graft deployment. The jejunum is divided close to the ligament of Treitz and again opposite the level of the third mesenteric branch. The isolated jejunal segment is then skeletonized upward, leaving the uppermost part in place for interposition. The conduit with vascular pedicle is passed without twisting and redundancy through the mesentery of the transverse colon and posterior to the stomach. The jejunum is anastomosed to the stomach and finally introduced through the esophageal hiatus into the mediastinum where the anastomosis with the proximal esophagus is completed. A free jejunal graft involves a microsurgical anastomosis of the graft vessels and suitable vessels in the chest. Jejunal conduit's befitting, isoperistaltic layout prevents mediastinal compression and its ability to clear acid reflux makes it conceptually attractive, but the high failure rate of this technique is due to the complex technique of harvesting the graft on fragile vasculature predisposing it to disastrous consequences.[42]

Colonic interposition

Colonic grafts are slowly losing its popularity; nevertheless, it is still a good alternative to stomach as a conduit. Either right or left colon can be used for interposition. Right-sided colon graft, ascending and proximal transverse colon, is based on the middle colic artery and if terminal ileum is used, then ileocolic branches are also preserved in the vascular pedicle. The most widely used procedure in the past was retrosternal esophago-colonic anastomosis involving right-sided colonic graft. The left-sided colon graft, left transverse colon, and proximal descending colon is based on the ascending branch of the left colic artery. The posterior mediastinal route is being used with better outcomes for interposing colon. The colonic graft, which essentially empties by gravity, may be positioned in an isoperistaltic fashion or anti-peristaltic direction if short vascular pedicle precludes isoperistaltic conduit layout. The colon is pulled up in the neck and end-to-side or end-to-end esophago-colic anastomosis is performed. To minimize or prevent acid reflux, the colo-gastric anastomosis can be done on the anterior or posterior wall of the stomach at the level of the cardia with an anti-reflux partial wrap of the stomach. Pyloroplasty or pyloromyotomy as a drainage procedure facilitates gastric emptying however, duodeno-gastric reflux is a possibility. Traditionally, these operations are performed through separate thoracotomy and laparotomy incisions or thoraco-abdominal single incision. Recently, laparoscopy complimented esophagectomy and colon interposition have been described in children with promising results.[33] Negative pressure in the thoracic cavity predisposes to the redundancy of colonic conduit; its known specific complication, leading to stasis, regurgitation, and dysphagia, which can be reduced by avoiding breach in the pleura and removing excess colon before anastomosis.

  Management of Failed Conduit Top

Evaluation and management of failed conduit is a difficult task. Early failure is due to graft necrosis in bowel conduits. The necrotic graft should be excised expeditiously and esophageal + feeding stoma re-established followed after few weeks new conduit can be planned. Redundant conduit is specific to bowel conduits seen after several years. If significantly responsible signs and symptoms are noted, then straightening and trimming of conduit or need to establish new conduit is necessary. Only radiological redundancy without symptoms does not warrant surgical intervention. Recalcitrant stricture is another indication for surgical re-intervention. It could be revision of anastomosis in the neck after excising small strictured anastomotic segment or long stricture mandating complete new conduit reconstruction. If previous conduit is in substernal location and if it is satisfactory, then the same can be rerouted through posterior mediastinum.

  Surgical Techniques to Retain Native Esophagus in Long Gap Oesophageal Atresia Top

There is no more consternating situation for any pediatric surgeon in the management of LGOA than retaining native esophagus. The key to success is the concept of multidisciplinary management approach at a center of excellence. Initial gastrostomy then delayed primary esophageal anastomosis within the first 2–3 months of life is commonly preferred approach with impressive outcomes. However, postoperative morbidity is high-strictures requiring multiple dilatations 58%, clinically significant gastroesophageal reflux 75%, need for anti-reflux surgery 50%. Other shortcomings of the delayed primary repair are prolonged hospitalization, inability to establish sham feeding with resultant oroversion after the establishment of esophageal continuity, cumbersome maintenance of upper pouch suction tube, which can lead to mucosal trauma, infection, electrolytes losses.[43],[44] Lee et al. reported similar experiences in children undergoing delayed primary anastomosis or esophageal replacement. However, long-term complications were more (86%) in OR cases as compared to 30% in the delayed primary anastomosis.[45]

Kimura and Soper's technique lengthens the upper pouch of esophagus by sequential advancement of the esophagostomy by mobilizing it over the chest wall; this could establish sham feeding and obviated the need to maintain an upper pouch suction tube.[46],[47] However, demerits of Kimura's technique are stricture and anastomotic leaks (30% and 80%, respectively) and requirement of multiple thoracotomies.[8] In contrast, traditional Foker's process is designed to induce rapid growth of the esophagus by external axial tension on the blind ending segments enabling delayed primary anastomosis within 1–2 weeks. Despite possible delayed primary anastomosis in more than 90% of cases its main disadvantages are disruption of the esophageal ends, risk of the anastomotic stricture (70%), anastomotic leak (50%), severe gastroesophageal reflux (60%), and requires multiple thoracotomies with its associated risks.[48],[49] The relative novel thoracoscopic Foker's approach in the management of LGOA may add an advantage by achieving maximal mobilization of esophageal ends under improved thoracoscopic vision.[50] Furthermore, the thoracoscopic approach can reduce the risk of infection and disruption of the esophageal ends compared with the traditional open Foker's process. In addition, if the primary anastomosis is not achievable after the application of the first tension sutures, a second internal traction sutures can be applied after further mobilization of esophageal ends. This method avoids risk factors of repeated thoracotomies and allows under vision application of tension sutures. In most instances, the esophagus is ready for the delayed primary anastomosis in 3–4 weeks.[51]

  Developing Concepts and Future Trends in Oesophageal Replacement Top

In times ahead, tissue engineering may be a potential therapeutic alternative to treat esophageal tissue deficit. Tissue engineering encompasses multidisciplinary approach of material engineering, gene therapy, physiology, and cell biology, aiming to regenerate native tissues. Studies in vitro and animal models have shown promising results in the creation of basic esophageal tissue using tissue engineering and regenerative technology. Two broad categories of tissues are available; cell-seeded scaffolds and cellular scaffolds; for facilitating the migration of autologous epithelial and smooth muscle cells to reconstruct new conduit. However, major challenges of longer length, cell-cell and cell-scaffold interaction, replicating peristaltic contractility, and vascular supply are yet unresolved. Complications such as leakage, strictures, infections, immune rejection, poor re-epithelialization, and poor muscle regeneration in the grafts suggest that still there is long time to go before extrapolating lab results into real life situations.[52]

  Conclusion Top

Esophageal substitution continues to be a daunting task in children. In general, irrespective of the OR technique, the long-term outcomes in the published data are much the same. In OA, it is preferable to do OR after 3 months and in corrosive strictures after 6 months of injury. It is well-nigh impossible to recreate all the features of a normal esophagus hence native esophagus is the best option. However, excessive, unfruitful attempts at preserving the native esophagus may ultimately prove deleterious to the child and family; therefore, surgeons caring for such children must remain familiar with various OR techniques.

The best-published outcomes are produced in large volume centers, thus reflecting the experience and passionate involvement of the surgeons involved in refining and perfecting their techniques. This is a typical indication for the creation of center of excellence or centralization of care of “difficult” esophageal cases for better outcomes, progress with research, and cutting-edge technology. Although good results can be obtained in a reproducible fashion in children using a variety of conduits depending on experience, currently, winds are blowing in favor of gastric transposition through posterior mediastinum due to relative simplicity of operation and long-term durability. Long-term follow-up of these children is mandatory as gradual changes in the function of graft, anastomotic stricture, intermittent dysphagia, less than satisfactory nutrition and growth, and the risk of metaplasia–dysplasia and malignancy are potential issues.

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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]


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