(Ed. Note: Dr. Humphreys is now in his upper nineties and still as sharp as a whip.  We were very excited to have his account of this story, as I am sure you will be, too.) He writes, "I am delighted that you were able to get in touch with Mr. Linsig and wonder if you could get him to write his memory of his childhood difficulties, as well as confirming the fact that for the past 45 years he has had no trouble eating.  That alone should encourage parents whose children may not yet have reached that stage.  I thought his story might be interesting reading for other parents of such children to learn that the solutions to these children's' problems took time and patience on the part of both the parents and the doctor.  Robert Linsig is the first person born with atresia of the esophagus to survive into adult life.  He is now sixty-one years old.  I first saw him in 1942 when he was brought to me at the age of seven.  Bobby Linsig's story, as I remember it, is as follows..."

  He was born in 1935 in Warwick, New York.  He was a vigorous baby, born at full term.  Nothing abnormal was noted at birth, but it soon became evident that he promptly regurgitated everything he swallowed.  Within a week of his birth a temporary gastrostomy was done, and feedings were begun with no difficulties. 

As he continued to tolerate gastrostomy feedings and regurgitate everything swallowed, his doctors decided to convert the temporary gastrostomy to a permanent one.  A flap of anterior gastric wall was used to form a tube placed through the abdominal wall at an angle so that feedings could be given by inserting a rubber tube through the opening, then withdrawing it until the next feeding without having gastric contents escape during the interval.  This method of feeding was successful.  As he grew, however, his mother became aware that when hungry, he often took food, put it in his mouth and chewed it up and swallowed it, only to regurgitate it a few minutes later.

At the age of two his mother decided to see whether she could take advantage of (and satisfy) his desire to eat normally by giving him a normal diet, let him eat it, then collect the regurgitated food and use it for his gastrostomy feeding.  It worked.  By the time she brought him to me five years later, he had learned to do it himself.  At this time, when ready for a meal, he would insert a tube into his gastrostomy with a large funnel at the outer end.  He would then eat his meal, regurgitating it at intervals into the funnel, from which it passed by gravity into his stomach.  This stomach, though socially unacceptable, was obviously, physiologically sound.  He grew and developed normally.

When I first saw him, he was a well-developed and nourished little boy, intelligent and cooperative.  It was obvious from his history that his esophageal normality could not be the commonest type of esophageal atresia, in which the upper esophagus ends blindly above the bifurcation of the trachea and the lower esophagus extends downward from an opening into the back of the trachea (a tracheo-esophageal fistula) to a relatively normal entrance into the stomach below the diaphragm.  When this anomaly is present, gastrostomy feedings (unless the fistula has been closed surgically) are always fatal because they pass upward into the lungs and cause aspiration and pneumonia.  Bobby had survived: therefore he had no fistula.

To verify this conclusion I took Bobby to the fluoroscopy room and, withsome trepidation, gave him a glass of barium contrast medium to swallow.  He did so without hesitation while the radiologist and I watched on the fluoroscope.  The barium passed normally over the larynx into the upper esophagus.  Then it filled, after several swallows, a large bulb-like sac of dilated esophagus just below the thoracic inlet in his upper chest.  Giving him an emesis basin, we watched him empty the sac or bulb by taking a deep breath, then bearing down with a closed glottis (the "valsaliva maneuver").  This emptied the esophageal sac neatly into the basin, without a drop of barium suspension entering the trachea.  We then filled his stomach with contrast material through his gastrostomy and were able to demonstrate that his lower esophagus ended blindly just above the diaphragm.

In 1942 I had been operating to remove cancers of the esophagus for some years.  The method was a staged procedure first described by Dr. Franz Totsk of the Lenox Hill Hospital in New York.  At the first operation the esophagus is removed from the chest; the upper end is brought out in the right neck, so that everything swallowed comes out through the esophagostomy opening, the lower end is closed.  A gastrostomy is then done, through which the patient is fed.  Swallowing is then restored, months later, by one or more operations.  A variety of methods could be used.

More recently, I had successfully adapted this method in dealing with a number of patients with atresia of the esophagus and tracheo-esophageal fistula.  At the first stage the newborn baby was operated on through the right chest.  Trying to stay out of the pleural cavity, the upper "blind" end of the esophagus was brought out in the neck and opened as an esophagostomy: the lower segment was separated from the trachea, the fistula or opening into the trachea was closed; and the open end of the esophagus was also closed and the esophagus replaced in the mediastinum.  A week later a gastrostomy was done for feeding.

Restoration of swallowing was not attempted for a year or more in order to let the organs grow and the baby gain enough vigor to withstand another major operation.  Ideally the ends of the esophageal segments could be united in one or more stages, but often the gap between the ends was too great: a variety of solutions had been proposed or performed by other surgeons.

I preferred a method using the upper small intestine (the jejunum) that I had used successfully in adults.  I decided that this method would be appropriate for Bobby Linsig.  I planned to first do the technically difficult operation to transfer a segment of the jejunum into the position of the esophagus in the chest, leaving the upper end closed, but close to the sac at the blind end of the upper esophagus: the transposed jejunal segment was then brought through the diaphragm and joined to the stomachs upper end with a good-sized side-to-side opening behind the stomach.  The segment then passed downward and through the transverse mesocolon, where the end of the segment coming from the stomach and duodenum was joined to the side of the long transposed portion that went up into the chest.  This forms a Y junction.  It was first described by a Swiss surgeon named Roux, so it is known as a "Roux-en-Y" procedure.  Because there is a risk that the circulation to the transplanted segment may fail, it is safer not to try to join the end of the intestine to the esophagus at this stage.  It would have to be done through a different incision in the upper chest, prolonging an already long procedure.  The second stage can be done about ten days after the first if there are no complications.

In Bobby's case the first operation went well.  But to my dismay, at the second stage I could find no trace of the end of the jejunum where it had been sutured in the upper chest.  I had to assume that the blood supply, at least to the top end, had been inadequate and that it died, or "sloughed."  It had not become gangrenous and formed an abscess, but could have simply atrophied and been absorbed into is lower portion.

This was a devastating anticlimax for the boy and his mother.  I tried to explain to them why I could not complete the operation that was to have enabled him to swallow, and I suggested further x-ray studies to see what had happened.  But his bitterly disappointed mother would not consent.  As soon as he was well enough, she took him home.

I did not see him again for six years.  Then he and his mother came back.  He had returned to regurgitating his meals into the funnel end of his gastrostomy tube, and it had served him well.  He was well nourished and had grown and developed normally.  They came back because they understood what I had told them that I had done at the first stage operation.  So, when he began to be aware that when he put ice cream or hot soup into his gastrostomy and then felt a sensation of cold or warmth in his left lower chest, it might mean that the transplanted intestine had survived in his chest after all.  A barium study soon showed that he was right.  With skillful manipulation it was possible to pass the tube entering his stomach through the gastrostomy, into the opening into the intestine on the back of the upper end of his stomach and to inject contrast material into a tangle of coiled intestine that lay above the diaphragm in his lower chest.  Evidently the transplanted segment of jejunum had not died—it had simply fallen away from where it had been sutured along his back and was lying on his diaphragm.  And when the stomach was then emptied, the transplanted jejunum promptly emptied itself by normal peristalsis back into the stomach.

The answer was now obvious.  Operating through the left chest I was able to untangle the coils of intestine, taking care to preserve its mesenteric blood supply, reattach it along the spine and open the closed upper end into the esophageal pouch in the upper chest.  He recovered uneventfully.  By the end of a week he was swallowing fluids with no regurgitation.  He then advanced first to soft, then solid food.  At Thanksgiving he was able to enjoy a full turkey dinner without using his gastrostomy for the first time in his life.

As far as I know, he never used his gastrostomy again, but at first he refused to let me close it.  It had served him so well throughout his childhood that I think having it there gave him a sense of security.  He knew that if anything went wrong with the new arrangement, he could always fall back into the old method of eating.  The small opening into which he had passed his gastrostomy tube for so many years did not leak.  He simply covered it with a small dressing.  He grew up, and in his late twenties he married.  His wife then persuaded him to return and let me close it.

As far as I know, he has had no difficulty in eating.  The transplanted small intestine does not, however, work exactly like a normal esophagus.  Normally we chew up a mouthful of food, mixing it with saliva; then we swallow a "bolus" of food.  This passes rapidly through the esophagus by muscular action: the esophagus is then empty and ready for the next mouthful.  When a mouthful of food is swallowed after an operation like Bobby Linsig's, however, it passes rapidly only through the upper esophagus into the dilated portion that was formerly the sac or pouch.  It is then pushed by muscular action of the esophagus through the opening into the transplanted intestine, through which it passes by slow peristaltic action into the stomach where the two were joined at the first operation.  This could be demonstrated by watching on a fluoroscope when he swallowed contrast material.  The material passed normally by peristaltic action into his stomach.  Although there is a continuing intestine through which swallowed food could pass down behind the stomach into the Y junction below the mesocolon without entering the stomach, little or no swallowed material goes this way, nor does the stomach empty through this section unless the patient tries to vomit.  This is difficult for him to do up the intestine in his chest, but his stomach can empty down the usually empty section of intestine behind the stomach.  The result, then, is that food cannot be eaten rapidly, and it must be well chewed, otherwise eating is no problem.  I have not seen Bobby Linsig for over thirty years, but I understand he is still doing well.
(December, 1996)

Dr. Humphreys died november 2001 at his home.