Introduction

Reconstruction with the patient’s own esophagus is recommended to obtain a satisfactory prognosis for congenital long-gap esophageal atresia (LGEA) [1]; however, primary repair is difficult for LGEA. Therefore, various lengthening techniques have been reported [1–5]. The standard approach used by most pediatric surgeons involves gastrostomy feeding, draining of the upper pouch, and allowing the esophagus grow over time with delayed primary anastomosis in 2–3 months [6]. In 1997, Foker et al. [1] reported an external traction technique applied to the two esophageal ends to stimulate rapid esophageal growth and achieve an earlier anastomosis. However, in some papers, this method has been reported to confer a risk of tearing the esophageal walls [2, 3]. We present our procedure of external lengthening for LGEA and its outcome, in comparison with other lengthening techniques (Howard and Kimura methods), at our hospitals.

Materials and methods

In our technique of external lengthening [7], two tiny polyvinyl chloride (PVC) tubes were fixed to each esophageal end to sandwich it without penetrating the esophageal lumens. Two nylon threads were passed through these tubes as traction threads (Fig. 1). The traction threads of the upper esophagus were pulled out of the thorax through the lower intercostal space, and those of the lower esophagus were either pulled out of the thorax or attached to the parietal pleura to prevent complications. During the traction period, the patients remained intubated and sedated but not paralyzed. We started the external traction for patients with type A LGEA after establishing enteral feeding through a gastrostomy. Because the enteral feeding is very important in neonates and the patients could not intake enteral feeding during the esophageal elongation and after anastomosis, we planned the timing at the beginning of the external traction after establishing enteral feeding. During the waiting period, daily bougienage of the upper pouch by using a Nelaton catheter was performed. Previously placed gastrostomies or new ones were liberally used in the postoperative period and closed as soon as possible.

Fig. 1
figure 1

Our modified Foker’s technique. Both the upper and lower esophageal ends were held and fixed with sutures between PVC tubes. Two nylon threads were passed through these tubes

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Five patients with LGEA underwent this external lengthening between 2010 and 2014 (group A), and 11 patients with LGEA underwent other lengthening techniques between 1990 and 2011 (group B). We compared the outcome between these two groups.

LGEA was defined as a distance between the upper and lower esophageal pouch of >3 vertebral units (VUs) at the first measurement. One VU is defined as one vertebral body together with one vertebral disc. The gap length at the first measurement was measured with boost pressure by inserting a Nelaton catheter into the upper esophageal pouch, and contrast medium was delivered into the lower pouch through the gastrostomy during fluoroscopy in type A LGEA cases (Fig. 2). In type C LGEA, the level of the lower pouch was estimated at the bifurcation of the trachea. The gap length at the beginning of external traction or reconstruction was measured with traction force at both the upper and lower esophageal pouches.

Fig. 2
figure 2

The gap length at the first measurement was measured with boost pressure by inserting a Nelaton catheter into the upper esophageal pouch, and contrast medium was delivered into the lower pouch through the gastrostomy during fluoroscopy in type A LGEA cases

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Results

Table 1 shows the five cases with external lengthening. The mean gestational age was 36 weeks, and the mean birth weight was 2001 g. There were four cases of type A and one case of type C. Four cases were prenatally diagnosed. The gap length at birth was from 3 to 5 VUs. All patients with type A had gastrostomy relatively soon after birth. The mean age and mean body weight at external traction were 20.2 days and 2172 g, respectively. The gap length at the beginning of external traction was from 1 to 4 VUs. The mean age and mean body weight at the esophageal reconstruction were 28 days and 2176 g, respectively. At the reconstruction, the upper and lower esophageal pouches were crossed in three cases, touched in one case, and 1 VU off in one case. The pathological findings of the upper and lower esophageal pouches sandwiched with PVC tubes in the three most recent cases were only artificially damaged muscular or fibrovascular tissue. Epithelial component was not observed. Table 2 shows the eleven cases with previous lengthening.

Table 1 Our cases with external traction
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Table 2 Our cases with previous methods
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Table 3 shows the comparison between groups A and B. The mean birth weight was 2001 g in group A and 2485 g in group B (p = 0.06), and the mean age at esophageal reconstruction was 28 days in group A and 227 days in group B (p = 0.03). Although primary esophageal anastomosis without myotomy was feasible in all patients in group A, myotomy was needed for primary esophageal anastomosis in half of the patients in group B. Moreover, a patient in group B eventually required gastric tube replacement.

Table 3 Our cases with external traction and previous methods
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Concerning postoperative complications (Table 4), anastomotic leakage occurred in none in group A and in six patients in group B (p = 0.03); anastomotic stricture requiring dilatation occurred in four in group A and in eight in group B; gastroesophageal reflux requiring fundoplication occurred in four in group A and in seven in group B; and refractory chylothorax occurred in one patient in each group. Stricture was defined as a narrowing of the anastomosis, giving rise to symptoms that required dilatations. Nissen fundoplication was performed if the patient had a recurrent stricture, and vomiting in combination with poor weight gain or pulmonary symptoms due to aspiration.

Table 4 Postoperative complications
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Excluding a patient in group A and two patients in group B who could not tolerate oral feeding because of neonatal asphyxia or complication of esophageal reconstruction, the mean age at the establishment of full oral feeding was 76 days in group A and 686 days in group B (p = 0.009). The PVC tubes separated from the esophageal ends during the external lengthening in three patients; however, the esophagus was effectively lengthened without perforation. Postoperative follow-up periods ranged from 8 to 64 months in group A. Regarding oral feeding, three of the five patients tolerated full oral feeding at the end of follow-up. A patient with 21 trisomy also tolerated oral feeding, partially supported by gastrostomy feeding.

Discussion

LGEA remains a challenge for pediatric surgeons. Because primary anastomosis is not possible in patients with LGEA, several surgical techniques are used to establish continuity. These include (1) primary repair under tension; (2) gastric mobilization with partial gastric pull up; (3) lengthening myotomies; (4) proximal and distal pouch stretching using various methods; and (5) esophageal replacement with gastric, colon, or jejunum interpositions. Although several techniques have been described for the management of a problematic distance between the upper and lower esophagus, the incidence of LGEA is so low that it is difficult for individual centers to gain large experience [8].

Previously, we selected bougienage for both the upper and lower esophageal pouches, or Kimura multistage extrathoracic esophageal elongation for LGEA with the goal of delayed primary esophageal anastomosis. Because both of these techniques took time to achieve primary anastomosis, we recently performed external lengthening techniques and tried to enable primary anastomosis at an earlier stage.

Foker and many others have described esophageal lengthening by using traction and subsequent anastomosis. Nasr and Langer [6] reviewed Foker’s external lengthening procedure and found it to be associated with a significantly lower risk of complications (leak, stricture, and gastroesophageal reflux) and with a significantly shorter time to definitive anastomosis.

Multiple simple suture traction has the problem of the suture cutting through the tissues [8]. This is because the total amount of the tissue included in the bite is much more than in the case of placing individual sutures, which reduces the chance of tearing. To prevent esophageal perforation and to keep the threads from slipping out, we applied a modified Foker’s external lengthening technique that involved holding and fixing the esophageal ends using PVC tubes without penetrating the esophageal lumen that could be detected on radiographs [7]. Foker pointed out that too superficial sutures could tear out under tension and too deep sutures could invade the lumen and cause infections [1]. In our cases with our modified technique, all esophagi were effectively lengthened without perforation even when the PVC tubes separated from the esophageal ends during the external lengthening. In addition, in the most recent case, we introduced the internal traction suture [9] only for the lower esophageal pouch with more moderate tension to prevent the PVC tubes from separating from the esophageal ends, and to prevent esophageal hiatal hernia.

The aim of our modification is to grasp each tip of the esophageal pouch broadly, firmly, and evenly, without damaging esophageal lumen. Therefore, we used a pair of PVC tubes sandwiching each tip of the esophagus, which may look like holding the tips with broad forceps. To elongate the esophagus effectively, the esophageal pouch should be sufficiently freed from the surrounding tissues, and continuous and slow traction should be applied to each tips of the esophagus. Comparing the methods of Howard’s bougienage and Kimura’s elongation, primary esophageal anastomosis had looser tension in external lengthening after the external elongation. Neither myotomy nor replacement was needed. The reported complications specific to Livaditis myotomy in the management of these cases are anastomotic leak, esophageal dysmotility, esophageal stricture, dysphagia, and life-threatening respiratory obstruction [10].

Although anastomotic leakage occurred in none of the cases with external lengthening, the risks of anastomotic stricture and gastroesophageal reflux were similar in these elongation methods. All anastomotic strictures were caused by gastroesophageal reflux in group A. Therefore, after fundoplication, the patients developed no more episodes of anastomostic strictures requiring dilatation by bougienage. Many reports have stated that patients with LGEA will usually need fundoplication and esophageal dilatation [11–13]. The technique should be improved to extend the esophagus further to prevent anastomosis with severe tension, and gastroesophageal reflux.

This is a retrospective observational study with a small group of patients. Nevertheless, our technique of external lengthening is less invasive [7] and enables primary anastomosis at an earlier age, which may facilitate oral intake.