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Atresia of the Esophagus: Technical Considerations in Surgical Management
Atresia of the Esophagus: Technical Considerations in Surgical Management I
i,
From the Surgical Clinic of 'I'he Children's Hospital of Philadelphia and the Harrison Department of Surgical Research, School of Medicine, University of Pennsylvania
i
C. EVERETT KOOP, M.D., Se.D. (MED.), F.A.CS. Surgeon in Chief; Professor of Pediatric Surgery
AMONG the congenital anomalies of the esophagus, by far the most common is atresia with concomitant tracheoesophageal fistula. In the classic form of this anomaly, the upper esophageal pouch is blind, hypertrophied and dilated and usually is long enough to extend into the mediastinum below the thoracic inlet, but usually does not descend as low as the carina of the trachea. The fistula between the trachea and the esophagus is at the level of the carina, and the lower esophageal segment is small in caliber because of disuse.
DIAGNOSIS
If one is interested in all of the factors which might contribute to lowering morbidity and mortality in the correction of this anomaly, diagnosis as well as operative techniques must be considered. The suspicious observer can be alerted to the presence of an atresia of the esophagus shortly after an infant's birth by the excessive amounts of mucus in the nasopharynx and by repeated bouts of cyanosis. The obstetric history of the mother usually includes hydramnios. It has been traditional to demonstrate the upper blind pouch by instilling into it an opaque material by means of a catheter. More than a decade ago, this was barium, which later gave way to an iodized oil and more recently has been superseded by one of the water-soluble opaque mixtures. None of these is necessary and each might be dangerous. It is very difficult to outline the pouch with any opaque material without spilling it into the 1387
1388
C.
EVERETT
Koop
Fig. 1. A, Coiled radiopaque catheter in dilated, hypertrophied esophagus proximal
to atresia. B, Lateral view of coiled catheter above atresia of esophagus. Air in bowel indicates presence of tracheoesophageal fistula.
tracheobronchial tree. Small amounts of barium are usually coughed up unless the baby is extremely debilitated, but large amounts of barium can produce atelectasis, collapse of a lung or even death. Iodized oil, an extremely tenacious material, slides into the tracheobronchial tree with the greatest of ease, where it coats the respiratory epithelium, diminishes the caliber of the airway and adds an insult to the aspiration pneumonitis which exists in almost every case, boding ill for the child. Water-soluble opaque media are probably least dangerous but are irritating. An opaque No. 10 red rubber catheter inserted into the blind pouch of an infant suspected to have atresia of the esophagus will curl up in such a way as to produce an unquestionable diagnosis on either the AP or the lateral film of the upper thorax (Fip;. 1). Such films should include the abdomen as well; the presence of gas in the stomach and beyond indicates the presence of a tracheoesophageal fistula and rarely a second alimentary tract obstruction may be shown. PREOPERATIVE PLANNING AND PREPARATION
Once the diagnosis has been made and operation has been decided upon, the planning of the procedure is the next consideration. Recent statistical analysis of a significant series of patients from the Hospital for Sick Children, Great Ormond Street, London, indicates that babies operated upon on the third day of life probably do better than those
Atresia of the Esophagus: Technical Considerations
1389
operated upon on the first or second day.! Thereafter, the mortality rises once again. Whether this will prove to be true in other series or not, certainly experience has proved that repair of esophageal atresia is not the urgent emergency that it was thought to be a decade ago. Except for unusual circumstances, it probably does not necessitate middle-of-thenight surgery, and the period between diagnosis and therapy provides an opportunity for effective use of antibiotics directed against pneumonitis, which is frequently a concomitant. In the period of preparation for surgery, a catheter should be placed in the upper pouch and constant suction maintained upon it. Inasmuch as regurgitation of gastric contents up the esophagus and through the fistula into the tracheobronchial tree seems even more damaging than aspirated mucus and saliva, it is well to keep the head of the patient slightly elevated so that gravity may work against this undesirable complication. Some of these youngsters get excessive amounts of air to their stomachs via the tracheoesophageal fistula, and some experienced surgeons perform a gastrostomy under local anesthesia before anything else to provide suction on the stomach and to prevent further aspiration of gastric contents. Anesthesia may very well be the keystone of success in repair of this anomaly. We believe that endotracheal administration provides so many safety factors and at the same time permits passage of a catheter into the upper esophagus that there is little to be said for alternate techniques. Because induction with bag and mask may force anesthetic gases and oxygen into the stomach, we prefer endotracheal intubation before induction. OPERATIVE PROCEDURE
Surgeons experienced in treating this anomaly are not agreed on the best method of approaching it. 2 Some prefer the retropleural approach after resecting a rib or portions of several ribs and feel that the preservation of the intactness of the pleural cavity in the event of a leaking anastomosis is worth the additional effort in exposure and the tedium of separating the pleura from the thoracic wall. Others, believing that the better exposure and shorter operating time made possible by a transpleural approach to the mediastinum compensates for the added risk of contaminating the pleural cavity in the event of a leaking anastomosis, approach the lesion transpleurally through an intercostal incision. After initial experience with the retropleural approach, we have, in general, preferred the transpleural technique, to which we have added two maneuvers which we believe contribute to the safety of the procedure. The mediastinal pleura is opened carefully and retracted meticulously. This permits it to be closed by interrupted sutures after
1390
C.
EVERETT
Koop
Fig. 2 Fig. 3 Fig. 4 Fig. 2. Tenting of upper blind pouch by stiff plastic catheter to designate apex. Fig. 3. Back wall of anastomosis completed. Fig. 4. Rubber dam drain placed retropleurally from lateral wound margin to mediastinum.
completion of the end-to-end anastomosis of the esophagus. Just before the thoracic cage is closed, a tunnel is made from the most posterior part of the separated ribs under the pleura in front of the posterior thoracic wall to the mediastinum. Through this tunnel, which becomes a retropleural fistula to the mediastinum on closure of the chest, is placed a small rubber dam drain. This connects the general region of the anastomosis with the skin by a route which does not traverse the pleural cavity. We have had the experience in using this technique of having an anastomosis leak, drain to the outside and never produce an inflammatory change within the pleural cavity as seen by x-ray. Once the mediastinal pleura is opened, the upper blind esophageal pouch can best be visualized and dissected from its bed by having the anesthesiologist pass into the pouch a relatively stiff plastic catheter. While the anesthesiologist maintains pressure on the catheter, the absolute apex of the blind pouch can be seen (Fig. 2) and a fine black silk suture can be placed through this most dependent portion of the upper pouch, to be used as a retractor. It is important in reference to the ultimate anastomosis that the most dependent part of the upper pouch be identified. When anastomoses have been carried out by suturing the lower esophagus into an eccentric position on the upper pouch, postoperative difficulties have ensued, the most difficult of which is a false diverticulum. Also, should the anastomosis require bougienage at a later date, it is not only difficult but dangerous to perform if the lower esophagus is not anastomosed to the exact center of the upper segment. Using the suture placed in the tip of the upper pouch as a traction suture, blunt dissection on all sides of the pouch will usually suffice to free it from surrounding structures except on the tracheal side. Occasionally, the association between the upper pouch and the upper trachea is intimate and separation must be started by careful sharp dissection. Rarely, a small fistula is encountered in this area which must be closed on both sides with interrupted sutures of fine silk if the defect is small.
Atresia of the Esophagus: Technical Considerations
1391
More will be said later concerning the technique of fistula closure in the trachea. The upper pouch may on occasion be so short as to be barely visible with its contained catheter at the thoracic inlet. There is no way of lengthening this pouch. The only technique we have found suited to this unfortunate circumstance is one in which the sutures for the ultimate anastomosis are all placed and then tied down later at one time. With the upper pouch separated from its surrounding structures and ready for eventual anastomosis, attention is turned to the lower esophageal segment. This may be found, if not readily visible, by identifying the usually prominent vagus nerve and seeking the esophagus immediately beneath (i.e., medial to) it. We make it a point never to touch either esophageal segment with forceps and particularly to handle the lower segment with the greatest respect because of its small caliber, lack of muscular development and frequent friability due to edema. A small right-angle hemostat such as the Jackson tracheostomy hemostat is an excellent instrument for dissection in this area. Once a tunnel has been made around the esophagus, a piece of broad tape rather than a suture which could cut the esophagus is placed in position as a sling. With gentle traction on this sling, sharp and blunt dissection can expose the esophagus up to the point of its attachment to the trachea. Rarely the esophagus makes its way into the right main stem bronchus, and occasionally its attachment to either bronchus or the trachea is broad, but usually the narrow lower esophagus joins the trachea almost at right angles much as a stove pipe joins the chimney. Before the esophagus is divided from the trachea, a stitch is taken close to the caudad junction of the two with a suture of 00000 black silk on an atraumatic needle. With fine sharp scissors such as those used by vascular surgeons, a cut is begun between the trachea and the esophagus, although the complete separation is not carried out at once. Choosing the right amount of esophageal cuff to leave for closure requires some thought. If not enough esophagus is left attached to the trachea for closure, a narrowing of the trachea may result that can cause a respiratory difficulty, emphysema and the loss of the patient. On the other hand, too long a cuff can result in vibratory breathing and the accumulation of mucus in the trachea. Enough esophagus should be left attached to the trachea so that when it is approximated with sutures, it assumes a contour rounded in conformity with the tracheal circumference. We prefer a running stitch, cutting the esophagus just ahead of the suture line so that there is not a wide open tracheal fistula at any time. Having completed the stitch from the lower to the upper end, we turn it and bring it back across the previous suture line so that a double row of running sutures is completed, and the final suture is tied to the initial long end left in place after the first stitch. Before closure is complete, it is helpful to take a culture of the tracheal secretions for bacterial
Atresia of the Esophagus: Technical Considerations
1393
pleted, a number of sutures being placed from the outside of the lower pouch into the lumen and from the lumen of the upper pouch to the outside. Keeping the catheter in place prevents picking up a piece of the opposite wall of the anastomosis and makes the procedure technically easier. Just before the final suture is placed, we ask the anesthesiologist to withdraw the catheter and to insert in its place a small polyethylene feeding tube with a blunt end and the perforations on the side of the tip. This is guided through the anastomosis under direct vision and is passed a sufficient length to deliver it through the esophageal hiatus into the stomach. The final suture is then taken and the anastomosis is complete. The mediastinal pleura is approximated over the anastomotic site with meticulous care. A tunnel is then constructed from the most posterior part of the wound under the pleura, between it and the thoracic wall to a point adjacent to the anastomosis in the mediastinum. Through this tunnel there is placed a fine strip of rubber dam which is then brought out through the wound to act as a drain should there be a leak at the anastomotic site (Fig. 4). The ribs are approximated with 000 chromic catgut and the thoracic wall is closed in layers, but not until a No. 14 red rubber catheter has been inserted in about the seventh or sixth interspace to act as a drain within the right pleural cavity. POSTOPERATIVE CARE AND FEEDING
Postoperatively, we do not plan a gastrostomy unless there is a specific indication. Gastrostomy has become very popular for almost any surgical situation in the newborn, but we believe that this additional surgical procedure should not be performed if it is not necessary. In premature infants of light weight, in a patient whose anastomosis was difficult because of friability of tissues or in a patient in whom the tension at the anastomotic line seemed excessive, a gastrostomy is electively performed. Later on in the postoperative course, should the feeding tube be pulled out by error, we may resort to a gastrostomy, but not always. Other indications are the presence of a leaking anastomosis and an eventual difficulty in getting adequate nutrition through the esophageal anastomosis. We prefer to nurse these babies postoperatively in a protected environment such as the Isolette. * Constant suction is not maintained on the chest catheter, but it is aspirated periodically by syringe and kept clamped at other times. There is usually no drainage after the second day, and the tube is usually ready for removal on the fifth day. If the gastrostomy tube is in place, constant suction is maintained on it; if not, suction is maintained on the polyethylene feeding tube for at least the first twenty-four hours.
* Air-Shields, Hatboro, Pennsylvania.
c. EVERETT Koop
1394
The starting of oral feedings, which usually begin with glucose and water, depends to some extent upon our confidence in the anastomosis. I have fed some of these babies as early as eight hours postoperatively when we were pleased with the type of tissue encountered at anastomosis and with the lack of tension in bringing the ends of the esophagus together. Usually, however, we give no feedings in the first twenty-four hours and start glucose and water by the polyethylene tube on the second postoperative day and feedings by mouth on the third, fourth or fifth day, depending upon our confidence in the anastomosis, the presence or absence of respiratory complications and the way the patient is tolerating the tube. Sometimes the lumen of the esophagus following anastomosis is so small that even the smallest caliber polyethylene tube effectively plugs the anastomosis. Under these circumstances, children may have excessive amounts of mucus which they cannot rid themselves of adequately and they require repeated aspiration. In such a case, it might be to the baby's advantage to remove the tube and let him dispoEe of his own saliva and mucus. The child's ability to do this is some indication also of when the edema at the site of anastomosis has subsided sufficiently to permit feedings by mouth. For those infants in whom a gastrostomy is in place, we use gastrostomy feedings on the second postoperative day, but it should be remembered that overdistention of the stomach by gastrostomy feeding can result in regurgitation up the esophagus with possible aspiration as a complication. Even with good anesthesia and a smooth surgical procedure, the fate of these babies rests largely on the skills of those concerned with postoperative care. Attention to increasing respiratory rate and aspiration of secretions because of this or the accumulation of mucus, close attention during the time of gastrostomy feedings, tube feedings or feedings by mouth and attention to the rate of flow of intravenous fluids may well requre the full attention of one nurse for the first several days postoperatively. These youngsters must be critically re-evaluated at short intervals, and it is mandatory that the surgeon or a member of a well trained house staff see them every two hours during the first thirty-six hours postoperatively. SPECIAL PROBLEMS
Special problems exist from time to time in the care of children with these esophageal anomalies. Many of them are born prematurely, and in most of these youngsters presented to us for therapy, we use the techniques which have been outlined. Occasionally, the life of a very fragile infant weighing under four pounds may be saved by doing a division and closure of the fistula with ligation of the upper end of the lower esophagus to prevent regurgitation. A gastrostomy for feeding and constant aspiration of the blind upper esophageal pouch to prevent over-
Atresia of the Esophagus: Technical Considerations
1395
flow into the trachea are mandatory until the infant weighs approximately five pounds, at which time an anastomosis may be attempted between the two esophageal segments. Some prefer to do the closure of the fistula in this instance behind the pleura, resecting a rib. Others believe that a quick transpleural approach is better in these tiny infants, particularly in view of the fact that there is no anastomosis to leak and produce contamination of the pleural space. Infants born with atresia of the esophagus, but without tracheoesophageal fistulae, present special problems because most of them have either no lower esophagus for an anastomosis or a lower esophagus so short that an anastomosis is not possible. This cannot be ascertained until the mediastinum has been explored from the right side, but the diagnosis can be suspected from the absence of gas in the stomach at the time of the initial diagnostic x-rays. In these circumstances, the upper esophageal pouch is freed in the same way as described previously and is left in situ. The thoracic incision is then closed and the child is turned to the other side in order that the blind pouch of the esophagus can be exteriorized above the clavicle by a cervical esophagostomy for the drainage of saliva. A feeding gastrostomy is then done, and this may well prove to be the most difficult part of the procedure. Children who have no tracheoesophageal fistula in the presence of atresia have a very small, contracted stomach, and the anterior gastric wall may not be much larger than the bulbous tip of a No. 12 mushroom catheter. In this situation, care must be taken in delivering the stomach; it is not possible to pull the stomach out of the abdominal incision, but rather one must expose it beneath such an incision for insertion of the catheter. In the unusual circumstances in which atresia of the esophagus is concomitantly found with another major gastrointestinal anomaly such as atresia of the duodenum or imperforate anus, the esophageal anomaly should probably be corrected first and then attention turned to the other one. In the presence of other small bowel lesions, the diagnosis may indeed not be known for several days after the correction of the esophageal anomaly, but even if the diagnosis is known, it may prove to be a wise choice to perform a gastrostomy for the purposes of gastric decompression and to postpone the surgical procedure on the bowel for several days. When imperforate anus is found in a patient with atresia of the esophagus, transverse colostomy is the procedure of choice except when a simple anoplasty will provide effective management of the anal lesion. REFERENCES 1. Aberdeen, E.: Seminar on Neonatal Morbidity and Mortality. Children's Hospital of Philadelphia, Feb. 1, 1962. 2. Koop, C. E. and Verhagen, A. D.: Early Management of Atresia of the Esophagus. Surg. Gynec. & Obst. 113: 103, 1961. 1740 Bainbridge Street Philadelphia 46, Pennsylvania
i,
From the Surgical Clinic of 'I'he Children's Hospital of Philadelphia and the Harrison Department of Surgical Research, School of Medicine, University of Pennsylvania
i
C. EVERETT KOOP, M.D., Se.D. (MED.), F.A.CS. Surgeon in Chief; Professor of Pediatric Surgery
AMONG the congenital anomalies of the esophagus, by far the most common is atresia with concomitant tracheoesophageal fistula. In the classic form of this anomaly, the upper esophageal pouch is blind, hypertrophied and dilated and usually is long enough to extend into the mediastinum below the thoracic inlet, but usually does not descend as low as the carina of the trachea. The fistula between the trachea and the esophagus is at the level of the carina, and the lower esophageal segment is small in caliber because of disuse.
DIAGNOSIS
If one is interested in all of the factors which might contribute to lowering morbidity and mortality in the correction of this anomaly, diagnosis as well as operative techniques must be considered. The suspicious observer can be alerted to the presence of an atresia of the esophagus shortly after an infant's birth by the excessive amounts of mucus in the nasopharynx and by repeated bouts of cyanosis. The obstetric history of the mother usually includes hydramnios. It has been traditional to demonstrate the upper blind pouch by instilling into it an opaque material by means of a catheter. More than a decade ago, this was barium, which later gave way to an iodized oil and more recently has been superseded by one of the water-soluble opaque mixtures. None of these is necessary and each might be dangerous. It is very difficult to outline the pouch with any opaque material without spilling it into the 1387
1388
C.
EVERETT
Koop
Fig. 1. A, Coiled radiopaque catheter in dilated, hypertrophied esophagus proximal
to atresia. B, Lateral view of coiled catheter above atresia of esophagus. Air in bowel indicates presence of tracheoesophageal fistula.
tracheobronchial tree. Small amounts of barium are usually coughed up unless the baby is extremely debilitated, but large amounts of barium can produce atelectasis, collapse of a lung or even death. Iodized oil, an extremely tenacious material, slides into the tracheobronchial tree with the greatest of ease, where it coats the respiratory epithelium, diminishes the caliber of the airway and adds an insult to the aspiration pneumonitis which exists in almost every case, boding ill for the child. Water-soluble opaque media are probably least dangerous but are irritating. An opaque No. 10 red rubber catheter inserted into the blind pouch of an infant suspected to have atresia of the esophagus will curl up in such a way as to produce an unquestionable diagnosis on either the AP or the lateral film of the upper thorax (Fip;. 1). Such films should include the abdomen as well; the presence of gas in the stomach and beyond indicates the presence of a tracheoesophageal fistula and rarely a second alimentary tract obstruction may be shown. PREOPERATIVE PLANNING AND PREPARATION
Once the diagnosis has been made and operation has been decided upon, the planning of the procedure is the next consideration. Recent statistical analysis of a significant series of patients from the Hospital for Sick Children, Great Ormond Street, London, indicates that babies operated upon on the third day of life probably do better than those
Atresia of the Esophagus: Technical Considerations
1389
operated upon on the first or second day.! Thereafter, the mortality rises once again. Whether this will prove to be true in other series or not, certainly experience has proved that repair of esophageal atresia is not the urgent emergency that it was thought to be a decade ago. Except for unusual circumstances, it probably does not necessitate middle-of-thenight surgery, and the period between diagnosis and therapy provides an opportunity for effective use of antibiotics directed against pneumonitis, which is frequently a concomitant. In the period of preparation for surgery, a catheter should be placed in the upper pouch and constant suction maintained upon it. Inasmuch as regurgitation of gastric contents up the esophagus and through the fistula into the tracheobronchial tree seems even more damaging than aspirated mucus and saliva, it is well to keep the head of the patient slightly elevated so that gravity may work against this undesirable complication. Some of these youngsters get excessive amounts of air to their stomachs via the tracheoesophageal fistula, and some experienced surgeons perform a gastrostomy under local anesthesia before anything else to provide suction on the stomach and to prevent further aspiration of gastric contents. Anesthesia may very well be the keystone of success in repair of this anomaly. We believe that endotracheal administration provides so many safety factors and at the same time permits passage of a catheter into the upper esophagus that there is little to be said for alternate techniques. Because induction with bag and mask may force anesthetic gases and oxygen into the stomach, we prefer endotracheal intubation before induction. OPERATIVE PROCEDURE
Surgeons experienced in treating this anomaly are not agreed on the best method of approaching it. 2 Some prefer the retropleural approach after resecting a rib or portions of several ribs and feel that the preservation of the intactness of the pleural cavity in the event of a leaking anastomosis is worth the additional effort in exposure and the tedium of separating the pleura from the thoracic wall. Others, believing that the better exposure and shorter operating time made possible by a transpleural approach to the mediastinum compensates for the added risk of contaminating the pleural cavity in the event of a leaking anastomosis, approach the lesion transpleurally through an intercostal incision. After initial experience with the retropleural approach, we have, in general, preferred the transpleural technique, to which we have added two maneuvers which we believe contribute to the safety of the procedure. The mediastinal pleura is opened carefully and retracted meticulously. This permits it to be closed by interrupted sutures after
1390
C.
EVERETT
Koop
Fig. 2 Fig. 3 Fig. 4 Fig. 2. Tenting of upper blind pouch by stiff plastic catheter to designate apex. Fig. 3. Back wall of anastomosis completed. Fig. 4. Rubber dam drain placed retropleurally from lateral wound margin to mediastinum.
completion of the end-to-end anastomosis of the esophagus. Just before the thoracic cage is closed, a tunnel is made from the most posterior part of the separated ribs under the pleura in front of the posterior thoracic wall to the mediastinum. Through this tunnel, which becomes a retropleural fistula to the mediastinum on closure of the chest, is placed a small rubber dam drain. This connects the general region of the anastomosis with the skin by a route which does not traverse the pleural cavity. We have had the experience in using this technique of having an anastomosis leak, drain to the outside and never produce an inflammatory change within the pleural cavity as seen by x-ray. Once the mediastinal pleura is opened, the upper blind esophageal pouch can best be visualized and dissected from its bed by having the anesthesiologist pass into the pouch a relatively stiff plastic catheter. While the anesthesiologist maintains pressure on the catheter, the absolute apex of the blind pouch can be seen (Fig. 2) and a fine black silk suture can be placed through this most dependent portion of the upper pouch, to be used as a retractor. It is important in reference to the ultimate anastomosis that the most dependent part of the upper pouch be identified. When anastomoses have been carried out by suturing the lower esophagus into an eccentric position on the upper pouch, postoperative difficulties have ensued, the most difficult of which is a false diverticulum. Also, should the anastomosis require bougienage at a later date, it is not only difficult but dangerous to perform if the lower esophagus is not anastomosed to the exact center of the upper segment. Using the suture placed in the tip of the upper pouch as a traction suture, blunt dissection on all sides of the pouch will usually suffice to free it from surrounding structures except on the tracheal side. Occasionally, the association between the upper pouch and the upper trachea is intimate and separation must be started by careful sharp dissection. Rarely, a small fistula is encountered in this area which must be closed on both sides with interrupted sutures of fine silk if the defect is small.
Atresia of the Esophagus: Technical Considerations
1391
More will be said later concerning the technique of fistula closure in the trachea. The upper pouch may on occasion be so short as to be barely visible with its contained catheter at the thoracic inlet. There is no way of lengthening this pouch. The only technique we have found suited to this unfortunate circumstance is one in which the sutures for the ultimate anastomosis are all placed and then tied down later at one time. With the upper pouch separated from its surrounding structures and ready for eventual anastomosis, attention is turned to the lower esophageal segment. This may be found, if not readily visible, by identifying the usually prominent vagus nerve and seeking the esophagus immediately beneath (i.e., medial to) it. We make it a point never to touch either esophageal segment with forceps and particularly to handle the lower segment with the greatest respect because of its small caliber, lack of muscular development and frequent friability due to edema. A small right-angle hemostat such as the Jackson tracheostomy hemostat is an excellent instrument for dissection in this area. Once a tunnel has been made around the esophagus, a piece of broad tape rather than a suture which could cut the esophagus is placed in position as a sling. With gentle traction on this sling, sharp and blunt dissection can expose the esophagus up to the point of its attachment to the trachea. Rarely the esophagus makes its way into the right main stem bronchus, and occasionally its attachment to either bronchus or the trachea is broad, but usually the narrow lower esophagus joins the trachea almost at right angles much as a stove pipe joins the chimney. Before the esophagus is divided from the trachea, a stitch is taken close to the caudad junction of the two with a suture of 00000 black silk on an atraumatic needle. With fine sharp scissors such as those used by vascular surgeons, a cut is begun between the trachea and the esophagus, although the complete separation is not carried out at once. Choosing the right amount of esophageal cuff to leave for closure requires some thought. If not enough esophagus is left attached to the trachea for closure, a narrowing of the trachea may result that can cause a respiratory difficulty, emphysema and the loss of the patient. On the other hand, too long a cuff can result in vibratory breathing and the accumulation of mucus in the trachea. Enough esophagus should be left attached to the trachea so that when it is approximated with sutures, it assumes a contour rounded in conformity with the tracheal circumference. We prefer a running stitch, cutting the esophagus just ahead of the suture line so that there is not a wide open tracheal fistula at any time. Having completed the stitch from the lower to the upper end, we turn it and bring it back across the previous suture line so that a double row of running sutures is completed, and the final suture is tied to the initial long end left in place after the first stitch. Before closure is complete, it is helpful to take a culture of the tracheal secretions for bacterial
Atresia of the Esophagus: Technical Considerations
1393
pleted, a number of sutures being placed from the outside of the lower pouch into the lumen and from the lumen of the upper pouch to the outside. Keeping the catheter in place prevents picking up a piece of the opposite wall of the anastomosis and makes the procedure technically easier. Just before the final suture is placed, we ask the anesthesiologist to withdraw the catheter and to insert in its place a small polyethylene feeding tube with a blunt end and the perforations on the side of the tip. This is guided through the anastomosis under direct vision and is passed a sufficient length to deliver it through the esophageal hiatus into the stomach. The final suture is then taken and the anastomosis is complete. The mediastinal pleura is approximated over the anastomotic site with meticulous care. A tunnel is then constructed from the most posterior part of the wound under the pleura, between it and the thoracic wall to a point adjacent to the anastomosis in the mediastinum. Through this tunnel there is placed a fine strip of rubber dam which is then brought out through the wound to act as a drain should there be a leak at the anastomotic site (Fig. 4). The ribs are approximated with 000 chromic catgut and the thoracic wall is closed in layers, but not until a No. 14 red rubber catheter has been inserted in about the seventh or sixth interspace to act as a drain within the right pleural cavity. POSTOPERATIVE CARE AND FEEDING
Postoperatively, we do not plan a gastrostomy unless there is a specific indication. Gastrostomy has become very popular for almost any surgical situation in the newborn, but we believe that this additional surgical procedure should not be performed if it is not necessary. In premature infants of light weight, in a patient whose anastomosis was difficult because of friability of tissues or in a patient in whom the tension at the anastomotic line seemed excessive, a gastrostomy is electively performed. Later on in the postoperative course, should the feeding tube be pulled out by error, we may resort to a gastrostomy, but not always. Other indications are the presence of a leaking anastomosis and an eventual difficulty in getting adequate nutrition through the esophageal anastomosis. We prefer to nurse these babies postoperatively in a protected environment such as the Isolette. * Constant suction is not maintained on the chest catheter, but it is aspirated periodically by syringe and kept clamped at other times. There is usually no drainage after the second day, and the tube is usually ready for removal on the fifth day. If the gastrostomy tube is in place, constant suction is maintained on it; if not, suction is maintained on the polyethylene feeding tube for at least the first twenty-four hours.
* Air-Shields, Hatboro, Pennsylvania.
c. EVERETT Koop
1394
The starting of oral feedings, which usually begin with glucose and water, depends to some extent upon our confidence in the anastomosis. I have fed some of these babies as early as eight hours postoperatively when we were pleased with the type of tissue encountered at anastomosis and with the lack of tension in bringing the ends of the esophagus together. Usually, however, we give no feedings in the first twenty-four hours and start glucose and water by the polyethylene tube on the second postoperative day and feedings by mouth on the third, fourth or fifth day, depending upon our confidence in the anastomosis, the presence or absence of respiratory complications and the way the patient is tolerating the tube. Sometimes the lumen of the esophagus following anastomosis is so small that even the smallest caliber polyethylene tube effectively plugs the anastomosis. Under these circumstances, children may have excessive amounts of mucus which they cannot rid themselves of adequately and they require repeated aspiration. In such a case, it might be to the baby's advantage to remove the tube and let him dispoEe of his own saliva and mucus. The child's ability to do this is some indication also of when the edema at the site of anastomosis has subsided sufficiently to permit feedings by mouth. For those infants in whom a gastrostomy is in place, we use gastrostomy feedings on the second postoperative day, but it should be remembered that overdistention of the stomach by gastrostomy feeding can result in regurgitation up the esophagus with possible aspiration as a complication. Even with good anesthesia and a smooth surgical procedure, the fate of these babies rests largely on the skills of those concerned with postoperative care. Attention to increasing respiratory rate and aspiration of secretions because of this or the accumulation of mucus, close attention during the time of gastrostomy feedings, tube feedings or feedings by mouth and attention to the rate of flow of intravenous fluids may well requre the full attention of one nurse for the first several days postoperatively. These youngsters must be critically re-evaluated at short intervals, and it is mandatory that the surgeon or a member of a well trained house staff see them every two hours during the first thirty-six hours postoperatively. SPECIAL PROBLEMS
Special problems exist from time to time in the care of children with these esophageal anomalies. Many of them are born prematurely, and in most of these youngsters presented to us for therapy, we use the techniques which have been outlined. Occasionally, the life of a very fragile infant weighing under four pounds may be saved by doing a division and closure of the fistula with ligation of the upper end of the lower esophagus to prevent regurgitation. A gastrostomy for feeding and constant aspiration of the blind upper esophageal pouch to prevent over-
Atresia of the Esophagus: Technical Considerations
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flow into the trachea are mandatory until the infant weighs approximately five pounds, at which time an anastomosis may be attempted between the two esophageal segments. Some prefer to do the closure of the fistula in this instance behind the pleura, resecting a rib. Others believe that a quick transpleural approach is better in these tiny infants, particularly in view of the fact that there is no anastomosis to leak and produce contamination of the pleural space. Infants born with atresia of the esophagus, but without tracheoesophageal fistulae, present special problems because most of them have either no lower esophagus for an anastomosis or a lower esophagus so short that an anastomosis is not possible. This cannot be ascertained until the mediastinum has been explored from the right side, but the diagnosis can be suspected from the absence of gas in the stomach at the time of the initial diagnostic x-rays. In these circumstances, the upper esophageal pouch is freed in the same way as described previously and is left in situ. The thoracic incision is then closed and the child is turned to the other side in order that the blind pouch of the esophagus can be exteriorized above the clavicle by a cervical esophagostomy for the drainage of saliva. A feeding gastrostomy is then done, and this may well prove to be the most difficult part of the procedure. Children who have no tracheoesophageal fistula in the presence of atresia have a very small, contracted stomach, and the anterior gastric wall may not be much larger than the bulbous tip of a No. 12 mushroom catheter. In this situation, care must be taken in delivering the stomach; it is not possible to pull the stomach out of the abdominal incision, but rather one must expose it beneath such an incision for insertion of the catheter. In the unusual circumstances in which atresia of the esophagus is concomitantly found with another major gastrointestinal anomaly such as atresia of the duodenum or imperforate anus, the esophageal anomaly should probably be corrected first and then attention turned to the other one. In the presence of other small bowel lesions, the diagnosis may indeed not be known for several days after the correction of the esophageal anomaly, but even if the diagnosis is known, it may prove to be a wise choice to perform a gastrostomy for the purposes of gastric decompression and to postpone the surgical procedure on the bowel for several days. When imperforate anus is found in a patient with atresia of the esophagus, transverse colostomy is the procedure of choice except when a simple anoplasty will provide effective management of the anal lesion. REFERENCES 1. Aberdeen, E.: Seminar on Neonatal Morbidity and Mortality. Children's Hospital of Philadelphia, Feb. 1, 1962. 2. Koop, C. E. and Verhagen, A. D.: Early Management of Atresia of the Esophagus. Surg. Gynec. & Obst. 113: 103, 1961. 1740 Bainbridge Street Philadelphia 46, Pennsylvania