Adult Chest Surgery

Chapter 38. Congenital Disorders of the Esophagus in Infants and Children 

 

This chapter presents the most common congenital malformations of the esophagus that require surgical correction in infants and children. Today, most of these entities can be corrected, and a child can lead a normal life after surgery. That was not true until 1939, when Logan Leven of Minneapolisand William E. Ladd of Bostonindependently saved a newborn with esophageal atresia on the same date! The operation consisted of dividing the tracheoesophageal fistula (TEF), marsupializing the blindly ending upper esophageal pouch, and feeding the baby temporarily through a gastrostomy. Later, a multistaged reconstruction was performed to make an antethoracic esophageal substitute, which was placed subcutaneously anterior to the sternum. The lower two-thirds of this conduit consisted of a Roux-en-Y loop of upper jejunum that bypassed the stomach and duodenum. The upper third of the conduit was a tubularized full-thickness graft comprised of skin and subcutaneous tissue. This was used to bridge the gap between the upper esophageal segment, which had been marsupialized in the neck, and the Roux-en-Y loop, which was brought up to the level of the upper sternum.

An important milestone in surgery for esophageal atresia was an insightful paper by Dr. Thomas Lanman, a colleague of Ladd, in 1940 describing a series of 32 esophageal atresia failures at Children's Hospital Boston.3Lanman predicted, "Given a suitable case in which the patient is seen early, I feel that with greater experience, improved technique, and good luck, the successful outcome of a direct anastomosis can and will be reported in the near future." This type of staged repair was abandoned in 1943 when Dr. Cameron Haight of Ann Arbor, Michigan, was first to report the primary definitive repair of esophageal atresia and TEF in the neonate.This was an important step in thoracic surgery for infants and children. Surgical treatments for other congenital esophageal anomalies were developed subsequently and are also described in this chapter.

ESOPHAGEAL ATRESIA AND TRACHEOESOPHAGEAL FISTULA

The spectrum of pathology in infants with esophageal atresia, with or without TEF, is illustrated in Fig. 38-1. These include esophageal atresia with distal TEF, without TEF, with proximal TEF, and with double (proximal and distal) TEF. Isolated TEF without esophageal atresia, so-called H-fistula, which usually occurs in the neck rather than midmediastinum, is also encountered. In most infants, esophageal atresia occurs with a single TEF. This form is observed in approximately 85% of affected infants. The upper esophageal pouch ends blindly, and there is a gap between it and the lower esophageal segment. The fistula from the lower esophageal segment usually enters the trachea just above its bifurcation. The blind upper pouch actually may overlap the nearby lower esophageal segment, or the two ends may be separated by a centimeter or more. Esophageal atresia without TEF is the next most common form and is seen in approximately 8% of infants.

Figure 38-1.

 

Types and frequency of esophageal atresia with and without TEF.

Diagnosis

A newborn in respiratory distress should be evaluated immediately for esophageal atresia with or without TEF. If there is no air in the abdomen, isolated "long gap" esophageal atresia should be suspected because a neonate normally has gas in the abdomen almost immediately after birth. Conversely, if there is an excess of gas in the stomach and intestine, TEF should be suspected. The presence of esophageal atresia can be ruled out by passing a small soft plastic catheter through the nose into the upper esophagus. Failure of the tube to pass into the stomach is diagnostic of esophageal atresia. One also can make this diagnosis by gently blowing air into the upper pouch to distend it under fluoroscopy. A very small amount of water-soluble contrast material is passed into the tube until the distal end of the upper pouch is visualized. Contrast material never should be injected without fluoroscopic guidance because it can overfill the pouch, causing aspiration. These babies often already have aspiration pneumonia before referral to the surgeon. In the rare case (see Fig. 38-1C or D ), an upper pouch fistula may be revealed when contrast material is put into the upper pouch. Note that the upper pouch fistula arises from the anterior wall of the blind upper esophagus, not its end, as occurs with the distal fistula. The clinical importance of this is that the unsuspecting surgeon in such a case may mobilize the upper pouch to an extent sufficient to perform the anastomosis while missing the presence of the second fistula. We have seen several such cases. A clinical clue to the presence of a second upper pouch fistula is the intermittent filling of the pouch with air as the anesthesiologist applies positive-pressure anesthesia through the endotracheal tube.

Other anomalies may be present in a neonate with esophageal atresia, most notably imperforate anus, which is a broad spectrum of anorectal anomalies and congenital heart disease. Appropriate consultation with a pediatric cardiologist may be indicated, particularly today, when the ideal time to repair many congenital cardiac defects, which are soon fatal, may be in the neonatal period.

Surgical Technique

ESOPHAGEAL ATRESIA

The surgical approach to esophageal atresia is made through the right chest in most patients. Rarely, if there is a right descending arch, it may be easier to approach the repair through the left chest. Neonatal thoracotomy, which transects the chest wall muscles, can result in impaired growth of the thorax on that side as the child matures. An incision that minimizes muscle cutting is that used by the late David Waterston at the Hospital for Sick Children, London, and is our preference. This is a vertical skin incision beginning just below the axilla and moving downward. The chest wall muscles are retracted upward to enter the fourth interspace. Spending a few minutes to bluntly separate the thoracic wall pleura off of the endothorax, keeping it intact, permits a posterolateral extrapleural exposure of the anatomy to be repaired. It is an important safety feature to locate the esophageal anastomosis extrapleurally to avoid widespread mediastinitis if a leak does occur.

There must be constant, close communication between the anesthesiologist and the surgeon because retraction of the mediastinal structures forward may embarrass cardiopulmonary function. Thus the anesthesiologist should warn the surgeon to release the hand-held retractors if there is any sign of cardiac slowing or if blood gas measurements show deteriorating levels. It is best to anticipate the possibility of these events, which proceed much more rapidly in a frail infant than in older patients. Anticipating a possible cardiac arrest in a baby is better than treating the actual event, which is not always salvageable. The surgeon who undertakes major chest surgery in a small baby must be gentle and work with an experienced pediatric anesthesiologist.

The azygos vein usually is divided to permit the approach to the esophagus, although we have done this surgery by mobilizing the azygos vein and maintaining it intact. If the vein is large and the baby is small, it is advisable to keep the azygos vein intact.

Early in the operation, the lower esophageal pouch should be identified. The vagus nerves will be seen coming from above and running along the sides of the distal pouch. They should be spared. As soon as the entry of the lower pouch into the back wall of the trachea is identified, it should be encircled with a ligature to stop the air blowing down from the trachea into the distal esophagus and from there into the stomach. If the stomach is already greatly overinflated with air from the fistula, we generally decompress it by placing a mushroom catheter into the anterior wall of the stomach through a minilaparotomy using a vertical incision in the midline approximately 2–3 cm in length. If a gastrostomy is used to decompress the stomach, we use the Stamm technique with two purse-string sutures of fine nonabsorbable material.

The tube should be brought out through a separate stab wound lateral to the laparotomy incision, with the added precaution of suturing the stomach to the abdominal wall. We have seen death occur when the stomach was not sutured and a leakage occurred at the gastrostomy site causing the stomach contents to spill into the upper abdomen. This is entirely preventable by anticipating this possibility. Regarding use of a stab wound, we have seen several infants die because infection occurred in the minilaparotomy incision when a tube traversed the wound. Infection caused a wide fistula between the stomach and the outside world, an entirely preventable event. Some of our teachers brought tubes through the main laparotomy incision. However, the surgeon who has seen that result in a major gastric fistula will prefer use of a stab incision for the tube.

We prefer simple ligation of the distal fistula, flush with the back wall of the trachea, and usually use a single ligature of 3–0 or 4–0 size. If the fistula is not ligated flush with the trachea, it leaves a tracheal diverticulum, which can cause intermittent episodes of pneumonitis. We have reexplored several infants in whom such a pouch had been left, another complication preventable by thinking of its possibility when doing the fistula interruption. An alternative method for closing the defect in the trachea is to close it with a row of fine interrupted sutures. The suture must have a tiny needle; otherwise, this closure can leak, whereas a ligature should not.

Mobilization of the distal esophageal segment must be done without injury to its thin, easily torn wall. Dr. C. Everett Koop would not allow a pair of forceps on the operative table when his resident mobilized the distal pouch. A pair of blunt, nontoothed forceps can be inserted into the open end of the distal esophagus, gently spreading it to permit mobilization without grasping its wall.

Conversely, the upper esophagus is usually thick-walled. It can be mobilized by passing a traction suture through its tip. This can facilitate, as the surgeon holds it first one way and then the other, to circumferentially mobilize the upper pouch. A useful maneuver, also of great help in mobilizing the upper pouch, is to pass into it a blunt-ended esophageal bougie, which is gently pushed down through the mouth by the anesthesiologist. Again, toothed forceps should be avoided to prevent injury to the infant's esophagus. Alternatively, a Bakes' common duct dilator appropriately bent can exert pulsion on the upper pouch, bringing it into view, especially if it is a high pouch located in the neck.

If the upper pouch seems to intermittently inflate as the anesthesiologist inflates the endotracheal tube, a search should be made for an upper pouch fistula. Mobilization of the upper pouch will disclose that it is often adherent to the adjacent back wall of the trachea. The two structures are separated by careful scissor dissection, staying off the back wall of the trachea, which is thin and easily entered. The esophagus is a much better developed structure because it has been obstructed in utero and is not easily transgressed. If a traction suture has been used on the end of the upper pouch, that part should be excised when the anastomosis is performed.

Early in the development of surgery for esophageal atresia, a two-layer anastomosis was used by many surgeons. The two-layer anastomosis consisted of a full thickness of the delicate distal esophageal opening to the mucosa of the upper pouch imbricating with a second layer of muscle from the upper pouch to the esophageal wall of the lower pouch. Today we prefer a single-layer anastomosis using the cut end of both upper and lower pouches. The back row of sutures is placed using 5–0 or 6–0 nonabsorbable sutures with the knots tied in the lumen of the two ends of esophagus. In practice, we place all those sutures first and defer tying them until the entire back row is in place. The assistant then grasps with large blunt forceps the two ends of esophagus, holding them gently together while the surgeon ties all the sutures with no tension. Most of the anterior wall sutures are then placed and tied simultaneously, again without tension on the ends courtesy of the assistant. The last two or three sutures then are necessarily placed such that the tie will be on the outside. At the completion of the anastomosis, we generally place two or three sutures in the muscular wall of the upper pouch to exert gentle traction downward to avoid tugging on the two ends of the anastomosis.

A small flap of mediastinal tissue can be used to cover the ligated and divided fistula at its tracheal end. A soft plastic catheter is placed in the mediastinal gutter well lateral to the anastomosis. Hence, if leakage occurs, it will track out along the tube. We anchor the tip of that tube in the upper gutter because many years ago we had a tube migrate into the esophageal lumen, which was a disaster.

If the postoperative course is uneventful, we obtain an oral contrast study at 7–10 days. If the anastomosis is intact, the chest tube is removed from the gutter, and feedings are begun by mouth. Some surgeons prefer to avoid using a gastrostomy tube and postoperatively leave a transanastomotic plastic tube through the nose into the stomach. However, infants breathe through the nose, and a transnasal catheter, in our opinion, can cause pulmonary complications. Therefore, we use them reluctantly. A surgeon who has endured a nasal tube for a week or more is not so apt to order one for someone else. This statement is backed up by ample personal experience!

The chest is closed with pericostal interrupted nonabsorbable sutures of appropriate size for the baby. Many of these infants are premature and require smaller sutures than are used for a full-term 7- or 8-lb infant. Our smallest esophageal atresia patient weighed 1 lb, 2 oz. If muscle has been cut, the ends are rejoined with interrupted sutures. The skin is closed with a running subcuticular suture.

Today, more than 90% of these infants should survive. Mortality is now limited mainly to those with other life-threatening anomalies or extreme prematurity, which itself carries considerable risk.

If postoperative contrast study shows narrowing at the anastomotic site, esophagoscopy and dilation are performed postoperatively. The safest way to dilate the esophagus in a small newborn is to pass a filiform catheter doubled back on itself into the stomach from above. A second filiform catheter is put into the stomach through the gastrostomy site. As the upper filiform catheter is passed into the stomach, pulling up on the transgastric filiform catheter usually will grab the tip of that passed from above, bringing it out of the gastrostomy site. Followers of increasing size then can be brought down from above through the anastomosis to dilate it. One must be careful not to overdilate the anastomosis, for we have had the unhappy experience of splitting a new anastomosis longitudinally, requiring reoperation.

There is a high (approximately 25%) incidence of gastroesophageal reflux in babies after surgery for esophageal atresia. In part, this may be related to excessive mobilization of the distal esophagus when the gap between the distal and proximal ends is longer than usual. Principally, however, the reflux is related to esophageal dysmotility, which is common in these patients. An antireflux operation may be indicated if gastroesophageal reflux persists. One of our patients developed carcinoma of the distal esophagus secondary to reflux at age 20 years.She was treated by distal esophagectomy with thoracoabdominal replacement using transverse colon on left colic pedicle (see Fig. 38-7). The patient is now 39 years old and in excellent health and swallowing normally.

LONG-GAP ESOPHAGEAL ATRESIA

Long-gap esophageal atresia (see Fig. 38-1B ) is a special problem. It is possible to stretch the two ends of the esophagus by various means. The upper pouch can be elongated by passing blunt esophageal bougies from above several times daily. The principal problem with leaving the upper pouch intact, instead of marsupializing it, is the risk of aspiration if it is not suctioned dry many times each day. Stretching the lower end is a more difficult problem. A bougie can be passed through the gastrostomy site, but that is not as satisfactory as dilating the upper pouch. Alternatively, a Bakes' dilator can be used with ultrasound guidance to visualize its passage into the lower pouch. The late Fritz Rehbein of Germany described a method that involved passing sutures through the ends in such a manner as to gradually bring them together.In 1976, we, along with Dr. Richard Hale of the National Magnet Laboratory at Massachusetts Institute of Technology, described an electromagnetic technique to bring the long-gap ends together.We put a metal bullet into each end of the esophagus and placed the baby in an electromagnetic field (Fig. 38-2). Intermittently, we turned the magnet on, pulling the bullets together until the blind ends of the esophagus were apposed. Although this was shown to be effective, reluctance of certain members of the nursing service to participate in this activity led us to discontinue this treatment. The objection raised was the need to keep the infant restrained during the treatment, which lasted 4–6 weeks. Therefore, for three decades we used colon esophageal interposition by the technique of Mr. David Waterston, with whom we worked in 1962. Professor Gunther H. Willital of Munster, Germany, has reported an extensive series of patients treated using electromagnetic bougienage.8

Figure 38-2.

 

Electromagnetic technique using bullets to bring the ends of the esophagus together in long-gap atresia.

TRACHEOESOPHAGEAL FISTULA WITHOUT ESOPHAGEAL ATRESIA (H-FISTULA)

Isolated TEF (see Fig. 38-1E ) often escapes clinical recognition during infancy. The history is usually one of intermittent respiratory infections. A contrast swallow with films in the lateral or oblique view can make the diagnosis. Sometimes the fistula is not seen, however, especially if the radiologist has scant experience looking for fistulas. Endoscopy can be diagnostic. Searching the back wall of the trachea with a 30-degree endoscope (this can be either a bronchoscope or even a cystoscope without water) will reveal the tracheal side of the fistula on the back wall of the trachea. It is usually located in the lower neck, not at the level of the carina, as in the most common type of esophageal atresia with TEF. A small catheter passed through the endoscope and through the fistula will prove its presence. A useful maneuver when the tracheal end of the fistula is not obvious is to place an endotracheal tube to intermittently inflate the trachea with positive pressure while searching the anterior wall of the esophagus for telltale signs of air entering the anterior wall of the esophagus. A drop or two of methylene blue placed into the tracheal lumen can further help to reveal its point of entry in the anterior wall of the esophagus.

The isolated fistula is exposed using a transverse cervical incision just above the clavicle. We pass a Fogarty catheter through the fistula, which runs obliquely from the trachea down to the esophagus. The Fogarty balloon is inflated and maintained in the inflated position by cross-clamping the upper end of the catheter to trap the air in the balloon. Gentle tugging on the catheter will show where the balloon is located with minimal dissection. It is easy to inadvertently injure the ipsilateral recurrent nerve when dissecting in the tracheoesophageal groove in search of the H-fistula. Awareness of the recurrent nerve should prevent its inadvertent injury. We have encountered two patients in whom the recurrent nerve "did not recur." Both had an aberrant right subclavian artery that arose as the last great vessel from the aortic arch and passed from the left mediastinum, behind the esophagus, and out the right thoracic apex toward the arm. Awareness of this rare anatomic entity, in which the recurrent nerve does not loop around the subclavian artery and then pass up the tracheoesophageal groove, is essential. If the surgeon thinks of this, that error will not occur. By having the anesthesiologist intermittently pull back gently on the Fogarty balloon, it is possible to feel it with a finger. This allows the surgeon to dissect exactly to the fistula without encircling the trachea or esophagus. The fistula is doubly ligated and divided. Although the ends are at a slightly different level, a wise precaution to prevent recurrence is to interpose tissue between the divided ends. This is a cardinal principle of closing any type of fistula, wherever it occurs. A strap muscle works well for this purpose. We divide the sternohyoid muscle at its upper end, and rotate the upper end of the muscle backward to cover the esophageal ligature. This takes only a few minutes and provides great safety against recurrence.The outlook after this surgery should be a normal baby. We have treated one patient who was 10 years old before the diagnosis was made.

There is another rare communication between the gut and the tracheobronchial tree that should be mentioned. The trachea and the primitive gut are adjacent to each other early in development. If there is a persisting communication between the two, as the gut lengthens, that communication can be stretched all the way from the neck down through the thorax, where it can arise from the jejunum or even the biliary tree. We encountered one of these cases, after the initial workup failed to reveal a communication. Repeating the barium study and following the contrast material down to the duodenum disclosed the occult communication. It ran upward and entered the right lower lobe of the lung. Using postural drainage for several years in this 5-year-old boy had been of no benefit in controlling his obvious intermittent aspiration pneumonitis and was exactly the wrong thing to do. By removing that bowel communication, we were able to stop his pneumonia. Severe pulmonary clubbing of the fingernails disappeared. When last seen 45 years ago, he was completely well.10

TRACHEOESOPHAGEAL CLEFTS

Diagnosis

A cleft should be suspected if an infant has respiratory distress, especially when synchronous with feeding. If a contrast examination is performed, it should be with a very experienced radiologist who will use a minimal amount of thin water-soluble contrast material. If a cleft is suspected when a catheter is passed, it is probably safer to make a diagnosis endoscopically under anesthesia rather than attempting to show it radiographically. We believe that this is difficult surgery and that experienced help should be sought for one of these rare lesions. Failure to respect this tenet is likely to be followed by recurrence.

Clefts between the trachea and esophagus are much rarer than simple tracheoesophageal fistulas. They can assume many anatomic configurations. Two are shown in Figs. 38-3 and 38-4. A small one may be difficult to see both radiographically and endoscopically. For recognition, some clefts require a high degree of suspicion on the part of the surgeon. At endoscopy, a cleft should be suspected if there is a ridge of heaped-up mucosa on the posterior wall of the larynx. The actual opening may be inapparent. A useful maneuver for identifying the cleft is to insert a catheter into the upper esophagus, and the surgeon can blow puffs of air into its upper end to distend the esophagus and open the cleft as air leaks from the esophagus into the trachea. Another useful trick is to insert a Fogarty balloon catheter through the endoscope, probing the possible cleft to demonstrate its presence. If it is a small fistula or an especially small cleft, inflating and gently pulling back on the balloon can reveal its exact location. The Fogarty balloon also facilitates finding its exact location when exploring through the neck with minimal dissection.

Figure 38-3.

 

A. Typical laryngoesophageal cleft. B. After division. C. Interposed muscle.

 

Figure 38-4.

 

A. Laryngotracheoesophageal cleft and lower tracheoesophageal fistula. B. Initial surgery in neonate. C. Late reconstruction at age 3.5 months.

When separating and closing a cleft, either congenital or posttraumatic, it is best to leave a margin of esophageal edges on the tracheal tissue. This will prevent the creation of tracheal stenosis by closing the trachea to itself yet will not compromise the esophagus when using a little of its circumference to augment the trachea. In one case (see Fig. 38-4), the child died after discharge home. We traveled to that hospital and participated in the postmortem examination, which was consistent with aspiration pneumonitis. Of considerable interest was the microscopic cross section of the trachea. It showed that the rim of esophageal tissue had assumed the histology of the adjacent tracheal tissue, complete with cilia.

In contrast to a small cleft is the very rare instance in which the cleft involves the full length of the airway from the larynx to the carina of the trachea. Surgical repair has been reported by Donahoe and Gee, the first surgical success in the world.11

We have preferred to approach these clefts through the lateral neck, exercising great caution to avoid injury to the recurrent laryngeal nerve in the tracheoesophageal groove. Some surgeons prefer an anterior approach by splitting the larynx and upper trachea in the midline to approach the pathology from inside the airway. Our preference for a lateral approach is predicated by our preference to insert a strap muscle between the closure suture lines. We have not had a recurrence when that maneuver was used (see Fig. 38-3).

We have seen acquired clefts in several patients after trauma. One was in a youngster with severe head trauma whose airway was intubated with a large endotracheal tube that lay next to a large esophageal tube that had been used for tube feedings. Two large tubes adjacent to each other in an unconscious patient can lead to pressure necrosis of the posterior wall of the trachea and anterior wall of the esophagus. We also have seen an inadvertent opening made in the posterior wall of the trachea and anterior wall of the esophagus by a surgeon attempting to do an emergency tracheotomy under harrowing circumstances. We believe that it is safer to first insert a bronchoscope into the trachea to secure the airway, provide positive-pressure ventilation, and palpate the trachea easily. Counting down to the second or third ring for insertion of the tracheotomy tube under direct vision in a non-blood-filled wound is safer than hurried tracheotomy with poor visualization. There are some patients with neck anatomy that does not easily allow passage of a bronchoscope. In such circumstances, positive pressure with one assistant holding the face mask firmly and another squeezing the anesthetic bag gives time for a nonhurried tracheotomy. We routinely pass a large suture through the cut edge of the trachea on each side, tie a button into the suture, and place it external to the wound on the neck. If the tracheotomy tube should become dislodged in the early postoperative period, the buttons can be pulled up, bringing the trachea into easy view, and the tube can be reinserted accurately. We have seen multiple deaths occur during "emergency tracheotomy" that could have been avoided by these simple measures that are routine for an experienced surgeon.

Surgical Technique

General surgical principles are important to keep in mind when closing any type of cleft or fistula, whether it is in the pelvis, the neck, or anywhere else. It requires identification, division, closure of both ends, and interposition of living, well-vascularized tissue to avoid recurrence (see Figs. 38-3 and 38-4). Depending on the operative field, this living tissue may be omentum or a tissue flap of adjacent tissue such as pleura, intercostal muscle. To our utter surprise, we have seen several fistulas in which the surgeon had applied cautery with the expectation that it would cause the opening to shrink and close. This maneuver generally just produces a bigger fistula!

HIATAL HERNIA

Diagnosis

In the 1950s, pediatric surgeons did not fully appreciate the large numbers of infants and children who had gastroesophageal reflux with or without herniation of stomach into the chest. Indeed, reflux was often called "chalasia." Those babies were often treated by propping them upright in their cribs to reduce the likelihood of reflux. In some, it abated spontaneously. In others, it did not. In recent years, newer drugs have controlled gastroesophageal reflux in many cases and controlled gastric acidity, which, with reflux, can be devastating to the esophagus and lungs. Gastroenterologists and surgeons do not all agree regarding if and when corrective surgery should be used. There are indications, however, that most would agree should prompt surgical correction. Persistent failure to thrive is a strong indication. Another is reflux esophagitis, which can stricture the lower esophagus to a degree that resection and replacement are needed. Recurrent aspiration pneumonitis is another potentially serious complication. We have seen it lead to bronchiectasis. Waiting too long and coping with severe complications is not "conservative therapy" but borders on malpractice. The physician who refers a child for an operation should know what kind of operation the surgeon performs, what the complications are, and what the success rate is. Surgeons select other surgeons for their own family based on these important parameters. Other people's children deserve the same consideration.

Surgical Technique

In recent years, since the advent of limited surgical exposure, many surgeons have elected laparoscopic fundoplication as their method for this corrective surgery. When one of us (WHH) was given a choice 2 years ago about the type of exposure he desired for aortic valve replacement, the reply was "whatever will allow you to do the best job." A median sternotomy was elected by that very experienced surgeon. We believe, as do many other older surgeons, that minimal-access surgery has been overused. Indeed, there are many young surgeons being trained today who have had little open operative experience. Unfortunately, patients often request minimal-exposure procedures, and some surgeons will freely admit they use such procedures to attract more patients than they might with open surgery. Long-term follow-up will be mandatory to assess the ultimate results of surgery, especially for cancer, when comparing modalities used by the surgeon, (i.e., open, laparoscopic, robotic, or hand-assisted minimal-access technique).

A single barium swallow often will fail to disclose gastroesophageal reflux. If the clinician thinks it is present, an acid reflux monitoring examination should be used, and often a repeat study by barium swallow is useful to see reflux. Laryngobronchoscopy may show red and edematous mucosa of the larynx and trachea.

We have used the following technique of hiatal hernia repair in children since 1959 with a high rate of success.12 A vertical midline incision is carried up just lateral to the xiphoid (Fig. 38-5). A Denis-Browne ring retractor provides excellent exposure. In performing a hiatal hernia repair, it is important to prevent evisceration of the bowel, which can cause adhesions and later intestinal obstruction. The attachments of the left lobe of liver are incised, folding the lobe under and retracting it medially to the right to expose the esophagus. The phrenoesophageal peritoneum is opened to expose the esophagogastric junction. The vagus nerves are identified, and the lower esophagus is encircled with a nonlatex rubber drain. Note that the assistant retracts the stomach downward and forward, using a gauze sponge to hold the stomach, not a clamp. The esophagus is mobilized for several centimeters while retracting it with a rubber tape. The crura are exposed and approximated loosely such that the sutures will not cut through the muscle. The gastrohepatic ligament is dissected free from the lesser curvature so that it can be sutured to the median arcuate ligament, which is the medial rim of the aortic hiatus. This maneuver was described by Dr. Lucias Hill.13 Attachments of the greater curvature and short gastric vessels are opened to prepare for fundoplication. The fundus is brought behind the esophagus. It is not a completely circumferential wrap. The esophagogastric junction along the lesser curvature is fastened to the median arcuate ligament using pledgets to help bolster the fixation. Note the use of many fine sutures to tack the esophagus to its hiatus. The anterior aspect of stomach is fixed to the undersurface of the diaphragm. We have not used a complete wrap as described by Dr. Rudolph Nissen.14

Figure 38-5.

 
 

Surgery for gastroesophageal reflux. A. Exposure of the cardia. Midline incision (inset). B. Exposing the gastroesophageal junction and encircling the esophagus. C. Crura exposed. Median arcuate ligament exposed (medial rim of aortic hiatus). Short gastric arteries divided. D. Crura closed; not too tightly! E. Fundoplication (not complete wrap). Prepare for Hill maneuver. F. Completed repair. This pediatric operation is similar to the Hill repair described in Part 4, Chap. 32.

REPLACEMENT OF THE ESOPHAGUS USING COLON

This is an area in which there are several equally effective approaches. Also, the pathology requiring colon replacement of the esophagus in childhood is variable; some patients are children with esophageal atresia that could not be repaired except by interposing a tube of bowel. In recent years, Anderson,15 Spitz and colleagues,16 and others have preferred to pull the stomach up into the chest. Indeed, the late Richard H. Sweet preferred the gastric pull-up in patients with esophageal carcinoma.17 He performed a supraaortic anastomosis of stomach to upper esophagus or an anastomosis as high as the pharynx for higher lesions. On the other hand, what might be best in an adult with a limited life expectancy may not be best in a child who, it is hoped, will live an entire life span with the repair a surgeon makes during infancy or childhood. We have seen several patients in childhood who develop severe reflux esophagitis with ulceration and stricture after a gastric pull-up procedure. Those patients were treated by returning the stomach to the abdomen, restoring gastrointestinal continuity below the diaphragm, and using a secondary colon esophagus with cure of the problem.

Caustic burns of the esophagus are also one of the prime indications for esophageal replacement. Most of these unfortunate children suffer because of adults not keeping caustic solutions out of their reach. Such operations had their advent in America in the mid-1950s. In Europe, especially Russia, this started earlier with the frequent ingestion of sulfuric acid by Russian children at winter's end. In many houses, a container of sulfuric acid was placed between the inner and outer windows to prevent fogging during the cold winter months. The hygroscopic properties of the sulfuric acid prevented moisture buildup between the inner and outer glass panes. In spring, when windows were opened, children got their hands on the acid with disastrous results.

Surgical Technique

In our initial experience with colon replacement, we used the right colon rotated upward on the midcolic pedicle and brought the cecum and terminal ileum to the neck for an easy anastomosis with the cervical esophagus. Generally, this segment of colon was brought up in the anterior mediastinum, beneath the xiphoid, behind the stomach, and out the thoracic inlet. The principal complication seen in these patients was late onset of tortuosity of the retrosternal colon. To straighten the colon in these cases, we used a median sternotomy approach.

In 1962, we adopted the technique described by Mr. David Waterston of the Hospital for Sick Children, London.18 It is shown in Fig. 38-6.19 The patient is positioned either with the left side straight up or partially upright. A wide prep and drape are performed, including the neck, left thorax, abdomen, and left arm, which is then covered with a sterile towel. In this fashion, it is possible to hold the arm upward intraoperatively for access to the chest or downward for access to the neck. A ninth interspace thoracoabdominal incision is made, transecting the costal margin to expose the upper abdomen. The diaphragm is incised circumferentially, leaving a small rim to close it at the end of the operation. This technique preserves the phrenic nerve, which is cut when the surgeon opens the diaphragm through its dome out to the costal margin.

Figure 38-6.

 
 
 

A. Colon esophagus operation. B. Mobilizing transverse colon over left colic artery. C. Colon into left chest, pedicle behind pancreas. D. Colon into anterior wall of fundus. E. Completed lower reconstruction. F. Typical upper anastomosis in neck. G. Paraaortic anastomosis in upper thorax.

 

A Denis-Browne ring retractor provides excellent exposure, as can several other types of retractors. Reaching across the abdomen, the right colon can be reflected medially. The hepatic flexure is divided, making certain that it is far enough to the right to provide adequate length to reach as high as necessary without tension. In our experience, the transverse colon, rotated upward on the left colic pedicle of blood supply, dividing the middle colic, will reach all the way to the pharynx if necessary.

The spleen, stomach, and tail of the pancreas are reflected medially to bring the colon up behind those structures through the esophageal hiatus into the mediastinum. The esophageal stump is removed. Earlier in our experience, we used the esophageal stump in some patients, anastomosing the lower end of the colon esophagus to it. However, in some patients the stump had reflux esophagitis, and in others congenital cartilaginous rests were found, leading to stricture. Cancer has been reported in a rudimentary stump, probably from severe chronic inflammation.

The straight left colon lies just behind the lung hilum, with left colic vessels tacked to the adjacent mediastinal tissues. In many patients we anastomosed the lower end of the colon esophagus to the back wall of the stomach. More recently, we have preferred to have it enter anteriorly because this may reduce the likelihood of reflux in the recumbent position. It is important to have a length of colon esophagus below the diaphragm to reduce the likelihood of free reflux. Reflux of gastric contents into a colon esophagus is better tolerated than reflux into the esophagus because the mucus glands of the colon provide some protection from gastric acid. Colon continuity is reestablished between the ascending colon and the splenic flexure. Note that the anastomosis is located at a distance from the anastomosis of the lower end of the colon esophagus to the stomach because a short section of the intervening colon has been removed. This avoids adjacent suture lines. Spleen, tail of pancreas, and stomach then are laid anterior to the left colic artery pedicle, on which there is no tension. In this retroperitoneal position we have not seen anything herniate beneath the pedicle.

In some cases, such as shown in Fig. 38-7, the replacement was limited to the lower esophagus. In others, the colon esophagus must reach the upper thorax to be anastomosed in the upper chest or neck (see Fig. 38-6F ). A maneuver used by Sweet facilitated a high paraaortic anastomosis. This involved tunneling upward beneath the chest wall muscles to make a second thoracotomy in the fourth interspace (see Fig. 38-6G ). In extreme cases, such as extensive caustic burns, the colon esophagus can be brought to the lateral pharynx.20

Figure 38-7.

 

A. Adenocarcinoma in a 20-year-old patient after original esophageal atresia repair. B. Resection and restoration of continuity.

A short segment of jejunum can be rotated upward to replace the lower esophagus instead of using a segment of colon. It may be less prone to reflux when placed isoperistaltically because of the small bowel's inherent peristaltic waves, which are more effective than those of the colon. Many of our Japanese colleagues are expert at bringing small bowel all the way to the neck in surgery for carcinoma of the esophagus. We feel that using the colon is inherently easier and less apt to have compromised blood supply than the delicate mesentery of small bowel in a young child.

Complications

The most common complication after colon esophageal replacement is spontaneous lengthening of the colonic segment in the lower chest, causing it to form a right-angle flexure as it turns forward to go through the esophageal hiatus. This can produce a functional partial obstruction. It is repaired by going through the lower chest to shorten it, being extremely careful with its blood supply, which comes up from the infradiaphragmatic retroperitoneal space.

An unusual complication seen in several patients was partial obstruction as the colonic esophagus enters the left thorax beneath the clavicle. Resection of the medial half of the clavicle to enlarge that aperture was curative in each patient. In one patient, part of the first rib also was removed.

In some patients, reflux of gastric contents can cause aspiration at night. Pyloroplasty to enhance gastric emptying can be helpful, and some patients will respond favorably to sleeping in a semiupright position. Other complications we have encountered include two minor neck anastomotic leaks in 32 patients and two upper strictures in a child with preoperative esophagitis from a prior high gastric tube. Dilation and steroid injection relieved the mild stenosis. Some upper gastrointestinal bleeding was seen in 8 of 32 patients long term, a paraesophageal hernia, gastritis from outlet obstruction (operated), peptic ulcer, alkaline gastritis, and unknown cause in two. Four of thirty-two had symptomatic reflux. Barrett's esophagus with development of esophageal carcinoma has been encountered in two teenagers who had gastroesophageal reflux after repair of esophageal atresia. We are convinced that persisting reflux requires careful monitoring. The patient shown in Fig. 38-7 had a well-differentiated adenocarcinoma at age 20 in 1988, which was treated by distal esophagectomy and proximal gastrectomy, as shown. Heineke-Mikulicz pyloroplasty also was performed to enhance gastric emptying after proximal gastrectomy. She is now age 39, in good health, and has a teenage daughter.

VASCULAR ANOMALIES THAT CAUSE TRACHEAL AND/OR ESOPHAGEAL OBSTRUCTION

So-called vascular rings of the upper mediastinum were described by Dr. Robert E. Gross and E. B. D. Neuhauser at the Boston Children's Hospital.3,21 Little has been added in the literature since these original descriptions.

Gross emphasized several tenets that are essential in the repair of these anomalies. Foremost is dissecting thoroughly all the vessels of the mediastinum before dividing anything. In recent years, some surgeons have advocated a thoracoscopic approach to these anomalies. We believe that this is potentially dangerous, particularly for the operator who has not had a great deal of experience with the deranged anatomy in these patients. A left thoracotomy is performed. Part of the thymus can be removed to give extra room in the superior mediastinum. Each of the vessels then can be traced to figure out the details of the case at hand. A small anterior arch is easy to expose and divide (Fig. 38-8). When the posterior arch is the one that requires division, it can be very dangerous unless great precaution is taken to have secure control of the far end of that small arch, which is deep in the operative field and well over to the right side of the mediastinum. It is safest to divide the end of the small arch that is closest to the surgeon, by oversewing the aortic wall and then gently retracting it out of the way. Only then is the short stump farthest from the operator safely oversewn. We would be very reluctant to do this surgery at the other end of a thoracoscope rather than in an open mediastinum. The same is true when dividing the ligamentum arteriosum, which goes between the left pulmonary artery and a right descending thoracic aorta (Fig. 38-9). Sometimes this ligament in fact carries some blood.

Figure 38-8.

 

Great vessel anomalies. A. Typical vascular rings with small arch anteriorly. B. Ring divided and ligamentum released.

 

Figure 38-9.

 

A. Right descending aorta after left ligamentum arteriosum. B. View from above. C. After release.

The diagnosis of vascular rings can be made by an experienced radiologist, together with an experienced surgeon, studying simple films. A barium swallow will demonstrate esophageal compression. A lateral film of the chest will show obliteration of the tracheal column as it passes through a vascular ring. An angiographic study seldom adds much that cannot be determined by the experienced eye. We have seldom resorted to angiographic studies despite the fact that they are much safer today than they were several decades ago.

Surgical Technique

Vascular anomalies of the great vessels in the thorax should be meticulously dissected to avoid injury to the esophagus or trachea Figures 38-8, 38-9, 38-10, 38-11, 38-12, and 38-13 illustrate the most common or important types that can cause compression of the trachea and esophagus. A double aortic arch with a small arch in front of the trachea and a larger arch behind the esophagus is illustrated in Fig. 38-8A. To relieve this vascular compression, the small arch is divided as shown in Fig. 38-8B. Also, the ligamentum arteriosum is divided to allow the left pulmonary artery to move forward and the descending arch to move back.

Figure 38-10.

 

A. Anomalous innominate. B. View from above. C. Aorta suspended anteriorly.

 

Figure 38-11.

 

A. Anomalous right subclavian artery. B. View from above. C. After release.

 

Figure 38-12.

 

A. Small posterior arch. B. View from above. C. View from behind. The posterior arch is always much shorter than shown in this illustration. The image is intentionally exaggerated to better show this pathology (see text).D. Ring opened.

 

Figure 38-13.

 

A. Pulmonary artery "sling." B. Pulmonary artery replanted with patient on cardiopulmonary bypass. Bronchial anatomy assessed and sleeve resection performed if needed.

The anomaly shown in Fig. 38-9 is a descending right aortic arch that is tethered to the left pulmonary artery by the ligamentum arteriosum, which passes behind the esophagus to the left pulmonary artery. It is curative to divide the ligamentum arteriosum, which "springs open" the compressive vascular ring. In this and all these types of obstructions, after the causative vessel is divided, the surgeon must visualize the formerly compressed trachea and/or esophagus to make absolutely clear that there is no residual problem. We have done reoperative surgery for several of these children when the first surgeon did not follow these precepts outlined 60 years ago by Gross.

A case involving compression of the trachea by an innominate artery that arises more distal on the aortic arch than usual is presented in Fig. 38-10. Note the vascular "crotch" between the medial wall of the innominate artery and the medial wall of the left common carotid artery. The ligamentum arteriosum is also short and tethers upward to the left pulmonary artery. Figure 38-10B shows this interesting anatomy in cross section. Relief of pressure on the trachea can be affected by thoroughly dissecting the great vessels of the arch and suspending the base of the innominate artery forward to the back wall of the sternum, as shown in Fig. 38-10C. This is done by placing Teflon pledgets on the anterior wall of the innominate artery so that fine sutures placed through the adventitia will not cut through. The ligamentum is also divided.

A case involving an aberrant right subclavian artery is illustrated in Fig. 38-11. Note that the right subclavian artery arises from the distal arch, passing behind the esophagus and traversing the apex of the right chest to emerge behind the clavicle. This is a common anomaly. It was described in 1794 in London.22 It is easily diagnosed with a barium swallow that shows the typical indentation of the back wall of the esophagus by the subclavian vessel that traverses upward and to the right, compressing the esophagus. There is a beaklike deformity on the esophagram in which the esophagus drapes over this vessel. It can be dissected free and divided in the thorax on the left side. Alternatively, in several cases we have dissected the vessel in the right lower neck anteriorly, following the vessel proximally into the posterior mediastinum, where it is ligated and divided, letting the ends retract away from the esophagus.

The right recurrent nerve to the larynx does not recur in these cases, where there is no subclavian artery around which it normally passes. Instead, the nerve enters high in the larynx from above. In several instances when operating for an isolated cervical TEF, we have seen and divided an anomalous right subclavian artery from the right neck incision. Obviously, the vessel must be secured and suture-ligated proximally before it is divided because retraction into the mediastinum through a short supraclavicular incision would be disastrous.

A double aortic arch with the smaller arch in back and larger one anteriorly is depicted in Fig. 38-12. This is the most dangerous type of arch to divide because the small posterior arch is usually very short, making it difficult to simply ligate and divide. We prefer to divide this arch between vascular clamps, leaving only a very short amount of the base nearer to the operator. That side then can be sewn over and over to control it, removing the proximal clamp. That segment of the large arch is retracted forward. If most of the posterior arch is retained on the far side of the mediastinum, as viewed by the surgeon, additional safety is afforded to oversew it deep in the mediastinum. If it should escape the clamp before it is oversewn, it can be very difficult to access the released segment deep in the mediastinum. Precaution is essential in dealing with this special anomaly.

Figure 38-13 shows an anomalous left pulmonary artery, often termed pulmonary sling. Note that the right pulmonary artery is normal, but the left pulmonary artery comes off superiorly, wrapping around the trachea and esophagus to then reach the left lung hilum. We believe that the safest way to approach this is through an upper sternal split, using temporary cardiopulmonary bypass. The anomalous left pulmonary artery is divided and reimplanted on the lateral aspect of the left main pulmonary artery. The trachea itself may be compromised by its severe compression. It may require sleeve resection. Happily, this is a very rare anomaly.

OTHER NONMALIGNANT CONGENITAL ESOPHAGEAL ANOMALIES

Achalasia

Achalasia is a rare esophageal motility disorder (see Chap. 24) that affects 5 in 1 million individuals of all ages. Only 5% of those are children under age 15.23 The underlying pathology, which consists of nonrelaxation of the lower esophageal sphincter and a markedly distended esophageal body, produces ineffective peristalsis and difficulty with swallowing. The presenting symptoms include weight loss, failure to thrive, superimposed dysphagia, and often regurgitation. On barium swallow, the esophagus has a wide body that tapers in conelike fashion to a very small distal segment. An air-fluid level is often observed. Esophageal manometry studies reveal ineffective and discoordinated peristaltic waves in the dilated esophagus and high pressure in the lower esophageal sphincter, where pressures exceed 40 mm Hg. As with any hollow viscus, the dilated walls fail to coapt, causing discoordinated peristaltic waves and an inability to propel a food bolus. This phenomenon can be seen in other conditions, such as megaureter and dilated colon in Hirschsprung's disease, where the lower colon does not relax. Achalasia is a progressive disorder. Medical therapy has included calcium channel blockers (e.g., nifedipine) and botulinum toxin injected endoscopically into the nonrelaxing lower esophageal sphincter. However, neither of these modalities has had satisfactory long-term results in children.24,25 Endoscopic balloon dilation was described more than 50 years ago by Gross26 as being effective in some children. Today, many physicians make several attempts to dilate the lower esophageal sphincter before referring a child for surgery.27 We believe that most young patients are best treated for this disorder surgically.

SURGICAL TECHNIQUE

The technique as performed in adults with achalasia is described in Chap. 26. Although the technique can be performed using open or minimally invasive technique, we prefer the laparotomy approach, as illustrated in Fig. 38-5. The procedure is performed with the tapered end of a Maloney rubber dilator inserted through the esophagus into the stomach. A soft nonlatex rubber drain is placed around the lower esophagus to free the esophagus through its hiatus. The myotomy begins several centimeters above the spastic segment, at or above the level of the inferior pulmonary vein, and is extended at least 1–2 cm onto the anterior wall of the stomach (cardia) to prevent postoperative reflux. After the myotomy, it is important to inspect the mucosa for evidence of perforation. This can be done by inflating the esophagus with air or saline through a nasogastric tube. The procedure then is followed as described in Fig. 38-5, including a pyloroplasty to prevent reflux. Some pediatric surgeons have performed this operation thoracoscopically. Experience with this approach is limited, and these children should be followed for postoperative gastroesophageal reflux. Our results using the thoracotomy approach have been satisfactory dating back to 1959, and reflux has not been a problem in our practice.

Of incidental interest, one of us has operated on three dogs with achalasia of the esophagus. The first was a female German shepherd puppy belonging to our family. The operation was as above. She died of recurrent aspiration several weeks later. Her brother, a 140-lb male, lived a normal life span. He sired several litters of healthy pups, but one had bowel atresia and one female had achalasia. She was operated on as above and lived a normal life span as a family member. This successful outcome was attributed to long-term loving postoperative care by the surgeon's wife, who fed the dog in an upright position for several months and gave supplemental feedings through a gastrostomy tube. The tube was hidden from the puppy, as well as her mother, by a dressing incorporating all the neighbors' discarded pantyhose. The third case was a Schnauzer who succumbed early postoperatively in the animal hospital where the procedure was performed by a pediatric surgical team.

Congenital Esophageal Stenosis

Congenital esophageal stenosis is a rare problem that occurs at a rate of 1 in 25,000 births.28 Thirty percent of these children have associated anomalies of the gastrointestinal tract, heart, or axial skeleton.29 The three known variants differ in embryogenesis and location. The first variant consists of a membranous web or diaphragm that typically is found in the middle third of the esophagus. It is thought to be an incomplete form of esophageal atresia and is the rarest type. The second variant is characterized by congenital fibromuscular hyperplasia. It is also found in the middle third of the esophagus and is the most common type, caused by submucosal smooth muscle and fibrous tissue proliferation and hypertrophy. The third form arises from ectopic tracheobronchial remnants, which are usually found in the distal third of the esophagus. This variant is thought to arise from tracheobronchial remnants improperly sequestered in the esophageal wall during the fourth week of gestation as the trachea separates from the esophagus.

Symptoms of congenital esophageal stenosis include both respiratory and gastrointestinal components, with vomiting, dysphagia, and feeding intolerance being most common. Reactive airway disease and recurrent tracheobronchiolitis are also seen often and may cloud the true nature of the problem unless the clinician has a high index of suspicion. Symptoms generally are progressive, and there is often a delay in diagnosis. A barium swallow should show the site of stenosis, but it may not elucidate the precise etiology. Esophagoscopy with endoscopic ultrasound is now used commonly to define the true nature of the problem. For esophageal webs or fibromuscular hyperplasia, endoscopic intervention with catheter-directed balloon-assisted dilation is the first mode of treatment. For many years, bougienage was used for dilation, but pneumatic balloon dilation techniques are now used more often.30

SURGICAL TECHNIQUE

Patients with tracheobronchial remnants or with fibromuscular hyperplasia and recurrent obstruction after multiple dilations should have segmental esophagectomy and anastomosis unless the segment is too long. Two layers of fine monofilament sutures are used to reconstruct the defect. Midesophageal lesions are best approached through a right thoracotomy, whereas distal lesions are best approached from the left side. If the lesion involves the intraabdominal esophagus, one should consider buttressing the repair with a partial fundoplication to prevent postoperative gastroesophageal reflux. Long-term outcomes have been excellent. Occasionally, a long-segment stenosis will require substitution with colon or small bowel.

Mediastinal Cysts and Esophageal Duplications

Several types of cystic masses occur in the mediastinum of infants and children. Some are neoplastic, and some are congenital.31 Teratoma, lymphangioma, and thymic cysts are found most commonly in the anterior mediastinum. Bronchogenic cysts are found more commonly in the middle mediastinum. In the posterior mediastinum it is more common to see esophageal duplications. Tumors of neural origin are found in the paravertebral region; sometimes these ganglioneuromas and neuroblastomas can extend into adjacent intervertebral foramina to involve the spinal canal. Some mediastinal masses are symptomatic, depending on their size and location. Others are picked up incidentally on routine chest x-ray.

Esophageal duplications represent 10–20% of all gastrointestinal duplications. They are rarely found in the cervical esophagus, yet we have seen upper airway obstruction caused by foregut cysts of the hypopharynx that block the inlet to the larynx.31 The mucosal lining may be entirely inappropriate for that level of the gastrointestinal tract where the duplication is found. This is an interesting feature common to all gastrointestinal duplications. The gastric mucosa can occur at any level from the neck to the pararectal region. Colonic mucosa similarly has been seen in duplications at any level of the gastrointestinal tract from the pharynx to the rectum.

There are two general types of esophageal duplications: enteric and intramural. Enteric lesions are most commonly tubular structures that result from incomplete separation of the primitive notochord from the endoderm during embryogenesis. If a defect such as a bony cleft is found anywhere in the vertebral column from the neck to the pelvis, one should consider a tubular duplication in the differential diagnosis. We have encountered several of these duplications traversing from the right thorax below the diaphragm up to the cervical region. These require formidable dissection in the neck, chest, and below the diaphragm, where the duplication may connect with the gastrointestinal tract or even the biliary tract. Intramural esophageal duplications are somewhat more straightforward. They are usually contained completely within the walls of the esophagus and probably result from incomplete vacuolization during development of the early esophagus. They seldom connect with the adjacent lumen, although if gastric mucosa is present, they can erode into the adjacent esophagus. Preoperative evaluation of these patients usually includes a barium swallow, which may demonstrate partial obstruction of the adjacent normal esophageal lumen from compression caused by the adjacent duplication. Chest CT scan after intravenous contrast material injection is also obtained routinely. If there is a vertebral defect, MRI also can add useful information.

During the surgical procedure, it is useful to gently pass a soft rubber bougie into the normal esophagus, if it passes easily, and then excise the adjacent duplication. The esophageal wall, which may be stretched over the duplication, is incised vertically, shelling out the duplication and taking care not to injure the underlying normal esophageal mucosa. In adults, it is not uncommon to follow a patient with a small esophageal duplication or an asymptomatic bronchogenic cyst with serial x-rays, although the anomaly can be removed easily thorascopically. In children with long life expectancy, however, there are several reasons these cysts should be removed.32,33 First, they may progressively secrete fluid and enlarge to sufficient size to produce respiratory symptoms. Second, they may become infected. Occasionally, a cyst may undergo malignant degeneration. If the cyst has a gastric lining, it may erode, causing hemorrhage or perforation. In the rare occurrence of a connection between the lumen of an esophageal duplication and the lumen of the adjacent esophagus, that opening should be closed with great care, before closing the esophageal muscular wall overlying the defect.

SUMMARY

Historically, major thoracic surgery in infants and children came to the forefront when Robert Gross became the first to successfully ligate a patent ductus arteriosus in a 7-year-old girl named Lorraine Sweeney. Gross was then the chief resident in surgery at the Boston Children's Hospital. His chief, William E. Ladd, was out of town. Ladd never forgave him for that surgical coup. Gross felt that Ladd would not have approved that risky venture. Today (2008), at age 78 years, Lorraine is alive and well and 70 years postoperative. This case exemplifies the aim of pediatric surgery, which is to make possible an entire life that otherwise might have ended in infancy or childhood.

EDITOR'S COMMENT

Many of the congenital esophageal disorders have been routinely addressed in infancy or childhood with great success. As a consequence, adult patients who had surgery as babies will occasionally present with long-term complications of the original procedure or anatomical challenges resulting from these procedures. For example, I have treated patients who have a substernal colonic conduit that was divided during an emergency CABG as an adult, as well as patients with diverticulitis in their intra-thoracic colon esophageal replacement. It is therefore incumbent on all thoracic surgeons to familiarize themselves with the various anatomic repairs of the congenital esophageal disorders. As for the current approaches to esophageal surgery in the pediatric population, these are evolving inexorably to less invasive methods, which require the same expertise and attention to details as the open approaches.

–RB

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