Esophageal ReplacementThe majority of esophageal procedures performed in infants and children are done for congenital esophageal atresia (missing portion of the esophagus) or acquired caustic strictures (scarring from lye or acid being consumed). With the former, the vast majority (92-97%) can be corrected without difficulty by primary esophagoesophagostomy (sewing the ends of the esophagus together). Successful esophageal anastomoses may even be performed in those few with "long gap" esophageal atresia, defined as a distance of > 3 cm between the proximal (top) and distal (bottom) esophageal remnants using various lengthening techniques. Preservation of the native esophagus is desirable and can be achieved in most cases. However, some patients with long gap esophageal atresia will require esophageal replacement. In addition, a number of those patients who are managed with primary repair will require an esophageal substitution as a result of complications of the primary procedure or development of severe gastroesophageal reflux, persistent stricture, and/or esophageal dysfunction. In those patients, preservation of the esophagus may be futile. Caustic injuries represent the second most common reason for esophageal replacement in children. Despite enhanced public education, safer packaging, and reduction in the concentration of sodium hydroxide in the most commonly used drain cleaner fluids, caustic injuries to the esophagus continue, especially in the less developed parts of the world, though at a lower rate, with the formation of scarring and strictures (narrowing) which can usually be managed with serial dilation (using tubes or balloons to widen the narrowing). However, 59% of severe caustic injuries will result in long and sometimes multiple strictures which are refractory to serial dilation. The only option in these patients for restoration of esophagogastric continuity is esophageal replacement. Alternatives for esophageal replacement in infants and children in the past have included a right or left colon interposition (running the large intestine from the back of the throat to the stomach), formation of a gastric tube (creating a tube from part of the stomach and swinging it up to the backing of the throat), and a jejunal interposition (running small intestine from the back of the throat to the stomach). All of these have advantages and disadvantages related to short and long term complications (Table I). In 1980, Atwell, et al., described the use of the stomach as a replacement for the esophagus in six pediatric patients, all but one of whom were newborns with congenital atresia of the esophagus. This was followed in 1987 with a review by Spitz, et al., from the United Kingdom, of similar gastric transpositions performed in 34 infants, of which 32 had esophageal atresia. Graft survival was 100% and outcome was excellent in 81% of the surviving patients. However, adoption of this technique in the United States has been slow, with few pediatric surgeons performing the procedure. In 1985, we switched from using colon interpositions to gastric transpositions for esophageal replacement in infants and children. Over the last 17 years we have performed 21 of these procedures at the C.S. Mott Children's Hospital and have been involved in an additional 20 performed at other centers. Technique of Gastric Transposition The intestines are prepared so that the colon is available should the gastric conduit prove to be unacceptable. The patient is placed on their back with the left chest elevated. The abdomen, chest, neck, and left arm are prepped and draped. An incision below the left rib is performed. If present, the gastrostomy is closed. Likewise, if a fundoplication has been performed, it is dismantled prior to proceeding with the transposition. The opening of the diaphragm around the esophagus is then widened to allow easy passage of the stomach and the lower esophagus is separated from the adjacent structures. A left neck incision is made and the esophagus is identified. If an esophagostomy is present, it is freed. If possible, the recurrent laryngeal nerve, which is involved in voice function, is identified and preserved on both sides. The esophagus in the neck is encircled and the neck, chest, and abdomen are freed. The esophagus in the neck is divided and a tube is attached to the lower portion of the divided esophagus, along with end of the chest tube, is delivered into the abdomen. In patients with esophageal atresia, in whom there has been no esophagus, a path is created between the neck and the abdomen. The junction of the esophagus and stomach is divided with a stapler. The highest point of the stomach is sutured to the chest tube and then brought up through the chest to the neck incision. The top of the stomach should be under minimal tension (Figure 1). The esophagus is then sutured to the muscles in the neck to prevent slippage of the stomach back into the chest. A hole in the top of the stomach is then created and a single layer anastomosis (joint) is performed between the top of the stomach and the upper end of the esophagus in the neck. A plastic drain is placed in the neck incision and the neck and the abdominal incisions are closed. An X-ray dye study of the area where the esophagus and stomach are joined is obtained on the seventh postoperative day (Figure 2). Experience Between 1935 and 2002, 554 patients with esophageal atresia were managed at the C.S. Mott Children's Hospital/University of Michigan Health System. A gastric transposition was performed in 21 of these patients. Gastric transpositions were performed in an additional 20 patients at other centers. The majority of the patients had a diagnosis of esophageal atresia (n=26) with other diagnoses including corrosive injury (scarring from drinking lye or acid) of the esophagus with severe stricture formation (n=8), leiomyomatosis (n=5), and refractory gastroesophageal reflux (n=2). Average age at the time of gastric transposition was 3.3 - 0.6 years (1 month - 15 years) for all patients and 1.7 + 0.4 for those with esophageal atresia (1 month -8.7 years). All of the long gap esophageal atresia patients with a tracheoeosphageal fistula (connection between the trachea and esophagus) were referred to the C.S. Mott Children's Hospital with a cervical esophagostomy after receiving their initial care at another institution. Thus none of these patients were candidates for primary repair of their esophagus. Since 1989, our approach has been to perform a gastric transposition in patients with Type A pure esophageal atresia without fistula, if indicated, as early as 1 month of age. However, only two such patients were primarily managed by us with this approach during that time period; primary anastomosis was performed in the majority of these patients. Five of the esophageal atresia patients had previous failed attempts at placement of a conduit: 3 colon interpositions and 2 substernal gastric transpositions. The latter two conduits constituted the only ones in our series which were placed just behind the sternum (breastbone). They were initially created at another institution and referred to us because of refractory strictures (narrowing). Of the three previous colon interpositions, two were performed at other institutions. There were 8 patients who underwent gastric transposition because of lye ingestion and the development of long strictures which did not improve with dilation. One of these patients was managed at the C.S. Mott Children's Hospital while the remainder were cared for at the various other centers. One of these latter patients had undergone a right colon interposition which failed. Five patients had leiomyomas (muscle tumors) of the esophagus. Finally, two patients with complicated gastroesophageal reflux were managed with removal of the esophagus and gastric transposition. There were no deaths and no loss of the gastric conduit despite multiple previous fundoplications and other chest operations in these patients. Small leaks from where the esophagus and the stomach were joined were noted in 15 patients (36%) and all uniformly resolved without intervention. Narrowing of this area (defined as requiring one or more dilations) formed in 20 patents (49%). However, none of these patients currently require dilations. Only 8 patients required more than 3 dilations and none of the anastomoses were revised. Immediate postoperative complications included 3 patients with vocal cord weakness, 2 with fluid around the lung, and 1 with pneumonia. Follow-up was from 5 months to 18 years with a mean of 6.5 + 0.8 years. Delay of passage of food from the stomach was initially observed in 5 (12%) patients. Inability to eat necessitating jejunal feeding at last follow-up was observed in 8 patients (20%) and was due to delay of food passing out of the stomach in three, severe neurologic impairment in four, and due to never having learned to feed because of to esophageal atresia in one patient. Eight of these patients under acceptable weight for their age. Only one patient demonstrated symptoms compatible with dumping syndrome (food leaves the stomach too fast and causes dizziness and/or diarrhea) which has resolved. There were no lung problems encountered and only one pneumonia occurred following the operation. Esophagitis (irritation of the esophagus from stomach acid) has not been noted in any of these patients. ReviewThe approach to the patient with long gap esophageal atresia is controversial and without a perfect solution. As such, a number of strategies for management have been developed. Most surgeons are in agreement that the original esophagus should be salvaged whenever reasonable. Studies have suggested that this can be accomplished in most newborns with long gap esophageal atresia. Mahour, et al. applied the technique of placing a rubber dilator in the proximal pouch once or twice daily along with periodic X-ray evaluation to see if the ends were growing together , demonstrating growth of both esophageal segments over a 4 to 13 week period. Successful joining of the two ends of the esophagus was achieved in all 12 of his patients. The incidence of leak, narrowing, and gastroesophageal reflux with this approach was high with fundoplication required frequently and removal of the narrowing required occasionally. Nevertheless, most patients ultimately did well. The high incidence (almost 100% in patients with long gap disease) of gastroesophageal reflux observed in these patients was often managed with a Nissen fundoplication, which can further aggravate the already present swallowing difficulties seen in these patients with a that is not working esophagus. In fact, esophageal replacement was performed in one patient in our series because the esophagus was working poorly following a Nissen fundoplication for gastroesophageal reflux. A number of techniques are used to aid in repair of the widely separated ends of the esophagus, but the esophagus may not work as well and the length may still be inadequate. Foker, et al., demonstrated successful approximation in those patients with esophageal atresia and gaps as long as approximately 7 cm by placing temporary sutures in the esophageal ends and applying increasing external traction over 6 to 10 days. Kimura, et al., used a process where the upper end of the esophagus was placed under the skin of the anterior chest wall and serially enlongated. Successful joining of the ends of the esophagus was achieved in all of his patients. Scharli recommends a dividing of the stomach in such a way as to allow the esophagus to lengthen. However, the Kimura and Scharli techniques have been done in a small number of patients with a large number of complications and even the necessity for subsequent replacement of the esophagus. Although the long gap esophagus can usually be successfully salvaged in a few, the ends cannot be put together or the esophagus does not work well. In children, whatever one uses to substitute for the esophagus must maintain excellent function for a lifetime. The colon (large intestine) interposition as initially described by Waterston et al. has been the most popular operation for esophageal replacement in children. However, the colon may die, may form an "S" shape and not work and the operation is complicated. An interesting solution to the absent esophagus is the reverse gastric tube which was popularized by Burrington and Anderson and Randolph.. The gastric tube remains narrow, does not form an "S", and serves most children well. However, gastric tubes may leak and narrow and the stomach may be small. In 1987, Spitz et al., reported their experience with 34 infants who underwent a gastric transposition to replace the esophagus. The authors demonstrated excellent results with a death rate of 9%. All of the deaths were lung-related and in patients with severe preoperative lung problems. In no patient did the stomach die. Four children developed narrowing where the esophagus and the stomach were joined which resolved with dilation. Two small leaks were noted and resolved spontaneously. An excellent result was noted in 25 children; in 4 there was mild difficulty swallowing. The majority of the children had excellent weight gain. A similar experience with the gastric transposition was reported by Marujo, et al. and Valente et. al. We have not experienced any lung symptoms in our patients. Early in our experience we used either the right or left chest to pull the stomach up into the chest. Subsequently, we have preferred the middle of the chest behind the heart. This is likely advantageous from a lung point of view since the stomach is confined within this area. We found removal of the esophagus with pulling the esophagus up to the neck to be safe despite a history of multiple previous operations in the esophagus, development of chest infections following failed attempts at replacing the esophagus with the colon, perforations (holes) in the esophagus as a complication of esophageal dilation, and the absence of a native esophagus, such as in pure esophageal atresia. Previous gastric procedures have often been considered a contraindication to gastric transposition. However, in our series, gastric transposition was performed safely in eight patients despite previous fundoplications for reflux. The vagus nerve, which affects the function of the stomach, is divided as a part of removal of the esophagus and gastric transposition. Likely as a result of the division of the vagus, we initially experienced delayed emptying of food from the stomach after gastric transposition in five patients. However, only three patients had this problem at last follow-up. Although we have not observed the presence of esophagitis in any of our patients, the long term risk of esophageal cancer is unclear. The only study addressing this issue is the one by Lindahl, et. al, in which they did biopsies on the esophagus in the neck in 14 patients more than two years following gastric tube replacement of the esophagus. Changes of the esophagus were found in ten patients, eight of which were confirmed histologically. As such, long term follow up with routine surveillance every 2-3 years is required, especially into adulthood. References
Suggested readings authored by the University of Michigan, Section of Pediatric Surgery
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