Acquired
Congenital
273 Caustic stenosis
Four malformations
Two long congenital stenosis
One long esophageal duplication
One achalasia
Two stenosis post-radiotherapies
One giant esophageal leiomyoma
Two stenosis post-viral infections (Herpes)
Two sequelae of epidermolysis bullosa
Two stenosis post-fungal infections (Candida)
On the opposite, since 1989, we have never performed an esophageal replacement, neither for a peptic stricture nor for an esophageal atresia born in our hospital. In our team peptic strictures are released with dilatations following anti-reflux procedures. The definition of a “long-gap esophageal atresia” as the “inability to achieve primary end-to-end anastomosis” is surgeon dependent. Most anastomosis of “long-gap esophageal atresia” can be done as delayed procedures, waiting sometimes for several months with a gastrostomy, as long as no cervicostomy has been done impeding from spontaneous lengthening. There is a strong correlation between too early procedures and complications including graft necrosis, anastomotic leaks, and sepsis [17–19].
Lee has compared delayed primary anastomosis with esophageal replacement with gastric tubes in a series of 44 patients with “long-gap esophageal atresia.” There was no difference in perioperative complications, but replacements had more long-term complications (86 %) compared to delayed primary anastomosis (30 %). Almost all patients experienced gastroesophageal reflux (GER) [20]. Pierro and coworkers, who has a large experience in esophageal replacements for long-gap atresia, very recently published a series of ten esophageal atresias without fistula with primary delayed anastomosis without replacement [21]. He concluded that the management of pure EA continues to be challenging, but the preservation of native esophagus is possible with significant morbidity, and the long-term outcomes are favorable.
13.3 Pathogenesis
(For more details, please refer to Chap. 16.)
Acids and bases can be defined as caustics, which cause significant tissue damages upon contact with the esophagus. Most acids produce a coagulation necrosis by denaturing proteins, inducing a coating coagulum that protects the underlayers from deeper penetration. Bases induce more severe injuries known as liquefaction necrosis, i.e., the denaturation of proteins together with a saponification of fats, which penetrate deep through the esophageal wall and can perforate.
The severity of the damages is related to several factors, including the pH, the concentration, and the volume of the agent. The contact time is of little interest as a lesion occurs within a few seconds. The physical form of the agent plays a significant role: the ingestion of solid pellets results in prolonged local contact time with the esophagus, thus deeper localized burns, while liquids generate superficial but more extensive lesions. For this reason, it is of major importance to refrain from drinking after pellet ingestion as it may induce both types of lesions.
Due to stagnation, lesions are more frequent and more serious at the level of anatomic narrowings of the esophagus (superior esophageal sphincter, aortic arch and left main bronchus at the level of T4–T5, and above the esophagogastric junction).
Like skin, the long-term effect of caustic esophageal burns is a hypertrophic scarring process, which can result in stricture formation. In addition, with the disappearance of the mucosa, the facing surfaces adhere to each other worsening the stenosis of the esophagus or occluding its lumen moving toward a fistula. Mucosal reepithelization is a slow process, usually not complete before 4–6 weeks. Not until a complete reepithelization, the inflammation continues, and granulation tissue comes to maturity. Thus, a stricture formation is detectable after 2 weeks and is definite by the fourth week. This is the best time to start dilatations.
If the muscular layers of the esophagus have been destroyed, they will not regenerate and be replaced by fibrous tissue. Even if the lumen has been kept open, the contraction waves will never overpass that point.
The caustic burn induces a shortening of esophagus and a motility disorder resulting in reflux and poor esophageal clearance, which adds a peptic stenosis to a caustic one evidenced by histology (O. Reinberg, unpublished). For this reason, all our patients under conservative treatment with dilatations receive proton-pump inhibitors (PPIs) as early as possible, even if their efficiency has not been proven [6, 9, 19].
13.4 Initial Treatment and Preoperative Preparation
(For more details, please refer to Chap. 16.)
The rate of stricture formation reported in literature varies from 2 to 63 % (!). About a month after caustic ingestion, the diagnosis of stenosis can be assessed by an esophagogram and an endoscopy, once the edema has gone. Then, according to the severity of the stenosis, a dilatation program can be started. The optimal frequency of dilatation is not well established in the literature, and our practice was to use a symptom-based approach, but an interval of 3 weeks seems appropriate in most cases.
Isolated short stenosis of the esophagus, i.e., 1–2 cm, can be treated by dilatations with good results. Long ones (more than 3 cm), multiple stenosis (more than 2), or those with a tracheoesophageal fistula cannot be solved by dilatations and require an esophageal replacement [5, 17]. However, the decision should not be precipitated as spontaneous improvement can occur within a few months until the lesions are stabilized. We have seen children with long narrow stenosis at 6 months that would have been candidates for esophageal replacement, who have been “forgotten” in their native countries. When they “reappeared” a year later, they only required dilatations of short narrow strictures. Subsequently, indications for esophageal replacements and their timing vary widely. As a result, children are often subjected to prolonged courses of dilatations prior to esophageal replacement or, conversely, may be exposed to unnecessary surgery [17]. A strong predictor of poor outcome was the delay from ingestion to the beginning of dilatations [17, 18]. Without improvement after 12 months of repeated dilatations, we consider doing an esophageal replacement as other authors [5, 22].
Esophageal replacements are major surgeries that require the child to be in a good nutritional condition. Esophageal strictures usually produce dysphagia for solids, liquids, or both, with slow and insidious progression of weight loss and malnutrition. If the stenosis is important with subsequent dysphagia lasting for more than a month, a gastrostomy should have been done. Most patients referred to us, even those with a previously done gastrostomy, were in poor nutritional conditions and must be placed under refeeding program before surgery.
The way the gastrostomy was done on the anterior stomachal wall is a major concern for the surgeon. When intending to replace an esophagus, the surgeon never knows which transplant he can use: if the gastrostomy has been placed too close from the greater curvature, he may face an interruption of the gastroepiploic artery, and the vascularization of the stomach can be compromised. When performing a gastrostomy for caustic stenosis, it is wise to place it far away from the greater curvature. In some cases, we used an interesting artifice suggested in 1974 by Papahagi and Popovici: when performing the gastrostomy, these authors ligated the middle colonic artery and sometimes the right one to stimulate the development of the left one, anticipating a possible transverse isoperistaltic colonic replacement [23].
A preoperative evaluation of the oropharynx and larynx has to be done preoperatively as associated lesions are not unusual: 15 % in our experience [5]. This initial evaluation should include vocal cord movements before surgery in the neck as paralysis can occur at the time of the caustic injury. We recommend the use of the consensual classification of benign laryngotracheal stenosis done by the European Laryngological Society [24]. The level of a preexisting tracheoesophageal fistula has to be defined by tracheoscopy. The length of the intact proximal esophagus above the first stenosis should be carefully measured to anticipate swallowing problems.
As other authors, we have abandoned mechanical preparation (enemas) [25, 26]. The day before surgery, we give an oral preparation of polyethylene glycol (= macrogol, 4 l/1.73 m2) through the gastrostomy (similar to those used before a colonoscopy), independently of the planned procedure, as we never know which transplant will be used.
13.5 Surgical Managements
13.5.1 Where Should the Esophageal Substitute Be Placed?
Choosing the appropriate route for esophageal replacement is an important decision.
The historic route was presternal (Fig. 13.1a) as the thorax could not be open at that time. Then the transplants were placed in the retrosternal position in a first procedure, and the native esophagus was removed in a second stage (Fig. 13.1b). We introduced the one-stage procedure in 1989, placing the transplant in the orthotopic position, i.e., in the posterior mediastinum, following a closed-chest esophagectomy (Fig. 13.1c) [27]. The path for an orthotopic plasty is straighter and shorter than that of the retrosternal route but requires removal of the native esophagus [28]. It avoids the two kinks at the upper thoracic inlet and at the reentry into the abdomen. It is our favorite procedure as it seems that the periesophagitis limits dilatation and redundancy of the transplant [2, 5, 27–29].
Fig. 13.1
The routes for substitute placement [2]: (a) historic presternal, (b) retrosternal, (c) orthotopic mediastinal posterior
However, in some circumstances, the retrosternal route had to be used when it appears impossible to dissect safely the esophagus or a previous transplant from the mediastinum. This is most often the case in the multiple redo procedures. In his series of redo procedures, Tovar had also to use the retrosternal route and even to go back to the old presternal path for one patient [30, 31]. It is easy to build a path behind the sternum in a space with very few adhesions. But with time, any transplant placed in this space will widen, especially if there is a narrowing at the distal end where it reintegrates the abdomen. This is more frequent in colonic transplants than in gastric tubes. Colonic transplants placed retrosternally have a strong tendency to become redundant, and we have had to tailor some of them. A gastric tube is more appropriate if the transplant is placed in the retrosternum.
13.5.2 Should We Remove the Native Esophagus and How?
There are two reasons to remove the native esophagus before an esophageal replacement: (i) to place the transplant in the orthotopic position, as mentioned above and (ii) because of the oncologic risk induced by the burned esophagus. The prevalence of malignancies, mostly carcinoma, is unknown but has been shown in several reports to range from 1.8 to 16 %, and the malignancies are known to take decades to develop. They were believed to be related to the abrasion of food intake on the burned esophagus. Subsequently, it was said that a disconnected burn esophagus did not bear that risk. Actually, no one knows the fate of a disconnected burned esophagus, and cases have been reported of carcinoma appearing on disconnected unused native esophagus after replacements [22, 32, 33]. For this reason, we remove as much as possible a native burned esophagus before replacement. However, a demucosed short segment of an abandoned disconnected esophagus is an acceptable risk.
In 1978, Orringer was the first to describe a blind esophagectomy without thoracotomy [34]. Since 1989, we introduced the one-stage orthotopic esophageal replacement following a closed-chest esophagectomy [27, 35]. The esophagus was removed through a left cervical incision after its transhiatal dissection by laparotomy without thoracotomy. A blind dissection by digitoclasy was performed in the middle part of the esophagus. At this level, adhesions to the major vascular structures and to the bronchi are the most severe and can lead to serious life-threatening injuries [35]. Some anatomical considerations on the vascularization of the esophagus are particularly useful when doing the hemostasis from the cervical opening and from the hiatus [36]. The greater danger remains at the level of the aortic arch and left bronchus where the most important adhesions are and which is the farthest point from skin incisions during the blind dissection. When total esophagectomy became too dangerous, we have abandoned some esophageal remnants at the level of the aortic arch after removal of the mucosa without subsequent narrowing of the esophageal substitute. Even after the experience in more than 200 cases, we considered this step as the most dangerous part of the procedure, showing 18 % of various complications. It allowed the esophagus to be totally removed in 45 % of cases and partially in 40 % [37] (Table 13.2).
In addition, several complications related to anesthesia can occur during blind esophagectomy. The most frequent were (i) endotracheal tube displacement during the dissection of the esophagus which requires tractions on it and through it and (ii) obstructions of the endotracheal tube or of the bronchi (mainly the left one) because of the mobilization of mucous plugs from the lungs during the esophagectomy.
For these reasons, we have tried to achieve esophagectomy under visual control without opening the thorax. Since 2006, we have used a standardized procedure through a laparoscopic transhiatal approach [38, 39]. This technique has been used by other surgeons since then [40]. Some cases of esophageal dissection using a thoracoscopy [41] or a combination of thoracoscopy and laparoscopy in children have also been reported [42–44]. The problem of the thoracoscopic approach is that it gives a lateral view of the esophagus that is hidden in the scarring process. It is safer to start the dissection from below by a transhiatal approach and to follow the intact esophagus up into the adhesions.
During the laparoscopic procedure, the child lays supine at the foot end of the table. The legs are wrapped in a “frog position” as for an anti-reflux procedure, and the table is tilted to a 30° anti-Trendelenburg position. In order to allow a good access to the esophagus, the right-hand port is placed in relation to the position of the gastrostomy, i.e., slightly inward and inferior to it. This will not only help the dissection of the esophagus, especially during dissection in the mediastinum, but also allow easier insertion of instruments by giving the appropriate direction to the mediastinum through the open hiatus. The esophageal diaphragmatic hiatus is enlarged by a 2–3 cm incision at 10 o’clock. Two large (0 or 2) trans-parietal monofilament threads are passed through the two crura from both sides of the patient and taken out through the skin. They allow a wide opening of the crura similar to the raising of a stage curtain. The transhiatal dissection of the esophagus is pursued under direct vision in close contact with the esophageal wall using a sealing device (Ligasure® LS 1500 Dolphin Tip or Maryland laparoscopic instrument by Covidien). The use of the harmonic dissector (Ultracision® by Ethicon) can be more dangerous as it heats in a very narrow field. Once the distal third of the esophagus has been freed, the liver retractor can be introduced into the mediastinum below the heart to allow a wider view of its major anatomical structures. A rotation of the 30° angulated camera helps to have a better view of both sides of the esophagus. This approach provides a clear view of the vagus nerves and facilitates their preservation. Should a pleural tear occur, a drainage tube is inserted under direct vision. The anatomical structures which run the greatest danger of being damaged during dissection are the left bronchus, whose soft posterior membrane usually adheres firmly to the esophagus, and the left brachiocephalic vein (innominate vein). The esophagus can be freed as far up as possible, usually one or two centimeters below the clavicle. With this technique of and more than 40 cases, no vascular or bronchial wound occurred, and the rate of the total removal of the esophagus raised up to 83 % without complication [38, 39] (Table 13.2). Moreover, it appeared that the delay to extubation and the length of stay in the pediatric intensive care unit (PICU) were shorter after laparoscopic transhiatal esophagectomy [38, 39].
Table 13.2
Esophagectomies: comparison according to the techniques used
Techniques | Blind dissection (1989–2006) | Laparoscopic transhiatal (2006–2014) | ||
|---|---|---|---|---|
N | 244 | 41 | ||
Mean ages | 5.9 | 6.0 | ||
Total esophagectomies | 111 | 45 % | 34 | 83 % |
Partial esophagectomies | 97 | 40 % | 5 | 12 % |
Failure | 36 | 15 % | 2 | 5 % |
Major accidents | 44 | 18 % | 0 | 0 % |
The cervical dissection of the esophagus requires the greatest care to avoid a tracheal tear or a lesion to the left recurrent laryngeal nerve. Preserving the most proximal centimeters of the native esophagus is crucial to avoid swallowing disorders.
A preexistent or preoperative tracheoesophageal fistula must be identified and occluded. The healing of such a suture requires coverage with a well-vascularized tissue because of the firm scarry processes in the mediastinum. For this purpose, several flaps can be used, such as pericardial flap, muscular flap taken from the intercostal muscles, or a flap from the latissimus dorsi in the most severe cases. However in some cases, we have left a part of the native esophagus after the removal of its mucosa and used it as a tracheal or bronchial coverage with success.
13.5.3 Which Transplant?
The esophagus can be replaced by a segment of the colon, the entire stomach, a gastric tube, or a part of the small bowel. However, none is perfect and can operate as a normal esophagus.
Gallo has performed a meta-analysis on 15 studies to compare three techniques for esophageal replacement of long-gap atresia in children: jejunal interposition, colon interposition, and gastric pull-up. The gastric pull-ups and colon interpositions appeared comparable regarding postoperative mortality, anastomotic complications, and graft loss. On long-term follow-up, the gastric pull-ups seem to be associated with a higher respiratory morbidity but fewer gastrointestinal complications than the colon interpositions. They were only two studies with jejunum and none with gastric tubes [45]. Loukogeorgakis and Pierro have published an extensive comparison of recent literature published over the last 5 years on the four main types of esophageal substitution that must be carefully read [46].
We are frequently asked which is our favorite transplant. We cannot answer this question and we used several of them [5] (Table 13.3). When intending to replace an esophagus, the surgeon never knows which transplant can be used: if the gastrostomy has been placed too close from the greater curvature, he may face an interruption of the gastroepiploic artery, and a gastric tube cannot be achieved. Should he plan a colonic transplant, a missing artery could make it impossible. Therefore, he must be able to adapt his technique to the patient’s condition and so must be aware of several techniques.
Table 13.3
Choice and place of 285 transplants
Transplant | N | Direction | N | Position | N |
|---|---|---|---|---|---|
Transverse colon | 234 | Isoperistaltic Antiperistaltic | 224 10 | Posterior mediastinum Retrosternal Posterior mediastinum | 221 3 10 |
Right colon | 8 | Isoperistaltic Antiperistaltic | 5 3 | Posterior mediastinum | 8 |
Colon mixt | 3 | Isoperistaltic | 3 | Posterior mediastinum | 3 |
Gastric tube | 36 | Antiperistaltic | 36 | Posterior mediastinum Retrosternal | 26 10 |
Jejunum | 4 | Isoperistaltic Antiperistaltic | 2 2 | Cervico-mediastinal | 4 |
13.5.3.1 Colonic Transplants
The colon is the most frequently used conduit to replace the esophagus; the transverse, ascending, or descending colon has been used, either in an antiperistaltic or isoperistaltic fashion. It offers the advantage of a segment of bowel with several possible vascular supplies that is long enough to be mobilized. Its width is approximately the same as the esophagus. Its length can be adjusted to the requirement [18, 22, 29]. This operation requires meticulous attention to technical details for a successful outcome. The use of the colon provides a good length of transplant and allows a tube of an appropriate diameter.
The operation is carried out through a midline incision from the xyphoid process to the umbilicus. The best transplant is taken on the transverse colon, vascularized by the left colonic artery and placed isoperistaltically (Fig. 13.2). Before ligating the unused vascular bundles, it is wise to generously mobilize the colon from the right to the left severing the gastrocolic ligament and to explore carefully its arteries. An efficient left colonic artery is missing in about 10 % of patients, and the anastomotic transverse colonic arcade can be absent. We check the quality of the chosen arterial supply by clamping the unused arteries during 10–15 min with atraumatic vascular bulldogs clamps. The superficial arteries must remain pulsating, especially those at the farthest end from vascular supply and the peristaltism be present. Once appropriate length is chosen, the transplant is prepared by severing the unused vessels while preserving long arcades. We use conventional ligatures and never coagulate them to prevent from vascular spasms. Once freed the transplant is cleaned and preserved in warm cloths avoiding any tension on its vascular supply.
Fig. 13.2
Isoperistaltic transverse colon vascularized by the left colonic artery
The colonic transplant has no efficient propulsive contraction and empties by gravity. However, in 1971, Jones first demonstrated on animals [47] and since then in humans [48] that an acid reflux in the transplant can induce a contraction that protects the colonic mucosa against acid aggression. When a reflux occurs, this intrinsic contraction, which can be reproduced with the amplitude of 15–20 mmHg for 45–50 s, rapidly clears the colon. For this reason, we believe that colonic transplants should be placed as far as possible in an isoperistaltic position to benefit from this self-protection.
If the right colon is used, it can be placed in an isoperistaltic fashion using a vascular supply from the middle colonic artery or antiperistaltically on the ileocolic artery. As the right colon is shorter than the transverse, the distal ileum is used with sacrifice of the valve to gain some extra length.
In some cases, we used an interesting artifice suggested in 1974 by Papahagi and Popovici: when performing the gastrostomy, these authors ligated the middle colonic artery and sometimes the right one to stimulate the development of the left one, anticipating a transverse isoperistaltic colonic replacement [23].
To bring the transplant to the neck, we use a large (40 mm) Penrose drain, the proximal end of the transplant being placed inside and sutured to it. This avoids any friction to its proximal edge when pulling it up. Before and after this maneuver, we check the arterial pulse with Doppler ultrasounds and the venous return as well. We handle the colon to find the best position for an optimal venous return. Some studies have been dedicated to the arterial supply of transplants but none about the venous return. However, it seems obvious that it is of great importance. Should compression or kinking occur on the drainage vein, the transplant could have a venous engorgement that may induce an ischemia with subsequent leak or stenosis [5].
We always perform the proximal end-to-end anastomosis using a single layer of full thickness interrupted resorbable sutures, with a V-shape incision of the proximal esophagus to make the colon width fit to its diameter if needed. In some cases if a short stenosis is present in the upper part of the native esophagus, we widen it using a Mikulicz procedure to avoid the anastomosis too close from the upper esophageal sphincter.
The distal cologastric anastomosis is performed on the anterior wall of the stomach by the upper third of the small curvature. The suture is done using two layers of resorbable stitches, with disrupted stitches on the seromuscular suture and a running one on the mucosa. A decompression tube is placed into the transplant though the gastrostomy together with a gastrostomy tube and a jejunal feeding tube.
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