Introduction

Esophageal atresia has been classically approached through a posterolateral thoracotomy. The disadvan-tages of such a thoracotomy have been recognized for a long time, for example winged scapula, elevation or fixation of the shoulder, asymmetry of the chest wall, rib fusion, scoliosis, and breast and pectoral muscle maldevelopment (Cherup et al. 1986; Chetcuti et al. 1989; Durning et al. 1980; Emmel et al. 1996; Freeman and Walkden 1969; Jaureguizar 1985; Schier et al. 2001; Westfelt and Nordwall 1991). Moreover chronic pain after thoracic surgery, at least in adults, is a serious problem and has been reported in more than 50% of patients (Perttunen et al. 1999; Rogers and Duffy 2000). Pediatric surgeons have tried to avoid classic postero-lateral thoracotomy. Brown (1952) made a vertical skin incision, detached the serratus anterior muscle, and opened the intercostal space. Soucy et al. (1991) intro-duced the muscle-sparing thoracotomy into pediatric surgery, while Bianchi et al. (1998) advocated transax-illary thoracotomy. More recently esophageal atresia has been approached in a thoracoscopically assisted way through a minithoracotomy (Robert and Hardy 1999), but true thoracoscopic correction was first re-ported in 1999 (Lobe et al. 1999). This child had an esophageal atresia without fistula. Thoracoscopic re-pair of an esophageal atresia with distal fistula was first reported by Rothenberg in 2000, while Bax and van der Zee published a first series of eight thoracoscopic repairs of esophageal atresia with distal fistula in 2002. In 2003 they published a series of 13 cases (van der Zee and Bax 2003). This chapter will only deal with the tho-racoscopic repair of esophageal atresia with distal fis-tula.

Preoperative Preparation

Before Induction of General Anesthesia

The preoperative preparation is no different from the preparation for open surgery. A chest X-ray with or without a radiopaque tube in the proximal esophageal pouch as well as an abdominal X-ray will confirm the diagnosis of esophageal atresia with distal fistula. The

The Thoracoscopic Approach to Esophageal Atresia with Distal Fistula Klaas (N) M.A. Bax and David C. van der Zee

Chapter 27

patient should be screened for other congenital mal-formations belonging to the VATER or CHARGE as-sociation, and consultation by a geneticist should be-long to the perioperative protocol. It would be nice to know preoperatively whether the aortic arch descends on the left or on the right in order to choose the side of the thorax to enter for the repair. If the aorta descends on the right, a left-sided approach would be preferable. A right descending aorta occurs in 1.8–2.5% of the cases (Babu et al. 2000; Bowkett et al. 1999). Ultra-sound examination seems to be a poor predictor. If a right descending aortic arch is noted during thoracos-copy, it is easy to change to the opposite side. Dextro-position of the heart is not a contraindication for a right-sided thoracoscopic approach.

After Induction of General Anesthesia

General anesthesia in our hospital is usually imple-mented with epidural anesthesia. The child is endotra-cheally intubated. End-tidal CO2 is measured continu-ously. An arterial line is inserted in the right radial ar-tery for invasive blood pressure monitoring and for blood sampling. Blood gases are determined regularly. A urinary catheter is also inserted.

The 10 French Replogle tube, which has been passed through one of the nostrils into the proximal esopha-gus, is loosened so that the anesthesiologist can push on it when required. Amoxicillin in combination with cla-vulanic acid and gentamicin is given perioperatively.

Positioning

Patient The patient is placed in a semiprone position on a short operating table with a pad underneath the right pecto-ral region so that the chest is tilted by about 15° to the opposite side. The table is tilted in about 15° reversed Trendelenburg ( Fig. 27.1).

Crew, Monitors, and Equipment The surgeon stands on the left side of the operating table with the camera operator to his or her left at the lower end of the table ( Fig. 27.2). The scrub nurse

200 Chest

Fig. 27.1. Patient position. The patient is placed in a semi-prone position on a shortened operating table. A pad is placed underneath the right pectoral region in order to tilt the thorax over 15° to the opposite side

Fig. 27.2. Position of the crew and equipment. The surgeon is standing in front of the patient’s abdomen with the camera operator to his or her left. Two monitors are used, one at each side of the head of the patient

Chapter 27 The Thoracoscopic Approach to Esophageal Atresia with Distal Fistula 201

stands at the right lower end of the table. The tower stands to the right of the patient’s head and the second monitor to the left.

A short (24 cm) 3.3 or 5 mm 30° telescope is used. The instruments are 20 cm long and have a 3 mm di-ameter.

Special Equipment

No special energy applying systems or special instru-ments are needed.

Fig. 27.3. Cannulae positions. The telescope cannula is lo-cated 1 cm below the inferior tip of the scapula and slightly anteriorly. The other two cannulae are inserted more proxi-mally, one more posteriorly and one more anteriorly

Technique

Cannulae

Cannula Method of insertion

Diameter (mm)

Device Position

1 Open 3.8 or 6 Telescope 30°, 24 cm long 1 cm below the inferior tip of the scapula and slightly more anteriorly

2 Closed 3.5 Surgeon’s left-hand instrument More posteriorly than the telescope cannula

3 Closed 3.5 Surgeon’s right-hand instrument More anteriorly and slightly more proximally than the telescope cannula

Figure 27.3 shows the positions of the cannulae. Some-times a 2 mm cannula is inserted behind the scapula in a closed manner for a 2 mm instrument to pull on the ends of anastomotic sutures or to pull on a vessel loupe around the distal esophagus during the tying off of the fistula.

All cannulae have siliconized sleeves to fix them to the skin and underlying fascia to avoid pulling out or pushing in. It is important to put the sutures not only through the skin but also through the underlying fas-cia, otherwise the cannula can still be pulled out be-cause of tenting of the skin.

Carbon Dioxide Pneumothorax

Carbon dioxide is insufflated at a pressure of 5 mmHg and a flow of 0.1 L/min. This is often not well tolerated in the beginning and close collaboration between the anesthesiologist and surgeon is needed. Increasing the ventilation rate while keeping the minute volume un-changed is helpful. Temporarily diminishing the pneu-mothorax pressure is also helpful. A saturation drop is counteracted by an increase in fractional intake of oxy-gen (FIO2). It is most important that the surgeon is not impatient. After a few minutes, the ipsilateral lung col-lapses and will stay so even when almost no pneumo-thorax pressure is applied. From then onward, the pro-cedure is usually much smoother than during a classic esophageal atresia repair. During thoracoscopic cor-rection no lung retractor is required.

202 Chest

Procedure

The procedure is only started when the baby has reached a stable condition. A magnificent view of the upper chest is obtained ( Fig. 27.4). The superior ca-val vein is seen anteriorly with the phrenic nerve lying on top of it. Usually two phrenic nerve branches merg-ing from the neck are seen. More posteriorly the right vagus nerve is clearly seen lying on the trachea. The distal part of the vagus nerve lies on the distal esopha-gus ( Fig. 27.5). The delicate vagal branches supply-ing the esophagus can be clearly seen. Some branches ascend to supply the proximal esophageal pouch as has been observed by Davies on postmortem specimens (Davies 1996). The proximal pouch is often only seen after a push by the anesthesiologist onto the Replogle tube. It lies more posteriorly against the vertebral col-umn. The distal fistula is located at the level of the azy-gos vein or above it ( Fig. 27.6). Its exact place can easily be identified as the trachea expands posteriorly at this level. The pleura above the azygos vein is opened longitudinally and the vein is freed circumferentially. The vein can then be severed between two intracorpo-really placed and knotted 5 0 polyglycolic acid liga-tures. Alternatively the vein can be coagulated with monopolar hook coagulation and then severed. For the same purpose the Ligasure could also be used but this requires a 6 mm cannula. With increasing experi-ence it is often not necessary to divide the azygos vein. The distal esophagus is mobilized as close to the tra-chea a possible and care is taken to avoid severing of vagal branches as much as possible. After isolation of the fistula, a 10 cm-long 5 0 polyglycolic acid transfix-

ing suture on a round-bodied needle is applied close to the trachea and tied intracorporeally ( Fig. 27.7). We nowadays apply the suture to the uppermost part of the fistula close to the trachea and bring the ends of the suture underneath the fistula. By doing so the ligature will stay close to the tracheal wall. In order to be able to introduce this suture through a 3.5 mm cannula, the needle has to be straightened somewhat. The distal esophagus is then transected a few millimeters distal to the transfixing suture. The end of the distal esophagus is slightly enlarged by a small cut into its dorsal rim.

Fig. 27.5. View of the midthoracic cavity behind the lung, which is collapsed. The distal esophagus with the vagus nerve on top of it is clearly seen, as is the azygos vein

Fig. 27.4. View of the upper right chest. The lung is collapsed. The superior caval vein is seen with two phrenic nerve branch-es, which unite, on top. More posteriorly the vagus nerve is seen

Fig. 27.6. The distal esophagus has been identified above the azygos, close to the trachea

Chapter 27 The Thoracoscopic Approach to Esophageal Atresia with Distal Fistula 203

Next the proximal pouch is mobilized laterally and posteriorly ( Fig. 27.8). No attempt is made to sepa-rate the pouch from the trachea as this interferes with its nerve supply (Davies 1996). Moreover we have the feeling that extensive mobilization does not add much length. A hole is then made through the end of the proximal pouch, using monopolar diathermy first and then hook scissors. The proximal esophagus should be well opened including the mucosa as a too small hole will result in stenosis. Because of the magnification ob-tained with the telescope there is a tendency to make the esophagotomy too small.

Suturing is started posteriorly in the middle of the esophagotomies. This first stitch is placed from the in-

side through the proximal esophagus and back through the distal esophagus from the outside. The knot will lie in the lumen. To overcome traction, a true square knot should be made and tumbled after which the knot can be slipped in place. Finally the knot is tumbled back by pulling on its ends in a 180° fashion. The next sutures can also be placed posteriorly in the same manner in front and behind the first suture. Care should be taken to include the mucosa into the bites. Once the poste-rior layer has been finished, the Replogle tube is re-placed with a 6 or 8 French nasogastric tube, which is passed through the anastomosis into the stomach until resistance is encountered. The tube is then pulled back a bit in order to avoid traction onto the anastomosis. The anastomosis is finished with a number of anterior sutures knotted extraluminally ( Fig. 27.9). It may be advantageous to insert a 2 mm cannula behind the scapula into the chest for introduction of a 2 mm for-ceps to grab previous suture ends and to pull on them in order to turn the anastomosis a bit and to expose areas that are otherwise difficult to access. Usually about ten sutures are inserted.

After finishing the anastomosis, the operative field is irrigated, and the cannulae are removed. The anes-thesiologist inflates the collapsed lung and the skin of the cannula holes are closed with adhesive tape. No chest tube is left behind.

Postoperative Care

A postoperative chest X-ray is made to evaluate lung expansion as well as the position of the endotracheal and nasogastric tube. The child remains mechanically

Fig. 27.9. The azygos vein has been divided between liga-tures and the anastomosis has been finished

Fig. 27.8. The proximal esophageal pouch has been mobi-lized posteriorly

Fig. 27.7. The distal fistula is intracorporeally ligated close to the trachea

204 Chest

ventilated for a few days and is then weaned from the ventilator. Feeding is started through the nasogastric tube on day 2. Oral feeding ad libitum is started when the child has been extubated and salivation has stopped. No routine contrast studies of the esophagus are per-formed. The child is discharged when on full oral feed-ing and doing well. As after open surgery, children with an esophageal atresia are regularly seen as an out-patient.

Results

So far 34 children with esophageal atresia and distal fistula have been treated thoracoscopically. Conver-sion was only required in one patient. This child was born after a gestation of 32 weeks with a weight of 1,230 g. It was feared that manipulation of the 6 mmcannula of the telescope in between the ribs could frac-ture the ribs above and below the telescope. Three pa-tients had dextroposition of the heart but this hardly interfered with the procedure. Three patients had a right descending aorta. In one the repair was done from the right, and in the remaining two thoracoscopy on the right was ended, the children were repositioned, and the repair was done from the left. None of the pa-tients died and operative time has come down to less than 2 h. There were four leaks, which all healed on conservative treatment. One leak was major and oc-curred in the second child of the series on the second day. There must have been a technical problem and an early repeat thoracoscopy might have been preferable. Twelve patients required dilation, most of them only once or twice. Four patients required an antireflux pro-cedure and three received an aortopexy. Two patients developed a recurrent fistula and these were repaired. All these secondary procedures have been performed endoscopically as well.

Many patients had several anomalies belonging to the VATER association and two patients had anoma-lies in the context of the CHARGE association.

The cosmetic result of the thoracoscopic repair was excellent in all patients (Fig 27.10).

Discussion

The thoracoscopic repair of an esophageal atresia with distal fistula has the great advantage of sparing the wall of the thoracic cavity. Moreover it allows for a much better view of the anatomy. In the case of a right de-

scending aorta, not much harm is done by entering the right chest and the child can be easily repositioned and scoped on the other side. Identification and limited dissection of the esophageal ends is easy. The most dif-ficult part of the operation is the suturing, which we do intracorporeally. Three patients in our series had a dextroposition of the heart. This did not make the op-eration much more difficult.

In open surgery, most corrections of esophageal atresia are done extrapleurally. There is, however, no evidence that a transpleural approach is less good.

It is often stated that the patient’s own esophagus is best. We do not share this view. In esophageal atresia without fistula, most of the esophagus is usually ab-sent. Both remaining ends can almost always be anas-tomosed, even thoracoscopically but this requires ex-tensive dissection and denervation of the ends. The morbidity in these patients is high. Recently a laparo-scopically assisted gastric pull-up procedure in a child with long gap esophageal atresia has been described (Ure et al. 2003).

Fig. 27.10. After a few months, the scars of the portholes are hardly visible

Chapter 27 The Thoracoscopic Approach to Esophageal Atresia with Distal Fistula 205

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