It is questionable whether in any of the reported cases the tissues of the obliterated portion of the esophagus have been entirely wanting. The matter could be settled in any instance only by careful, and often microscopical, study of the posterior wall of the trachea, since the tissues of the esophagus are firmly attached to this region and often apparently lost in it.

Happich has collected only II cases of this partial obliteration of the esophagus without fistula, viz., those of Marrigues, Lallemand, Brodie, Durston, Röderer, Toujan, Lichty, Shattock, van Cruyk, Weill and Pehu and Marsh.

We may add the case of Steel, 3 cases of Kraus and the 2 cases recently reported by Phillips, to one of which we have just referred.

(7) Obliteration of a Portion of the Esophagus with Tracheoesophageal (or Bronchoesophageal) Fistula.-This is by far the most frequent malformation found. Of Mackenzie's 63 cases of esophageal malformations, 43 were of this nature, and Happich has tabulated carefully 59. Detailed enumeration of these may be obtained in the bibliography of these two writers. We have found published in the last six years 14 additional instances, 12 of them since the appearance of Happich's monograph. These are, viz., cases of Kirmisson, Renault, Villemin, Patron, Keith and Spicer (4 cases), Dickie, Guyot, Fischer, Spicer, and Giffhorn (2 cases). Another case very briefly reported by Guillemet probably belongs to this category. In addition, there are 2 cases published by Dam and by Wunsch respectively, in which there was no autopsy made, but in which an obstruction was discovered by the use of a sound, and which possibly belong here also.

Malformations elsewhere in the body are very liable to occur in combination with this defect (28 of Happich's 59 cases), as, in fact, with congenital defect of the esophagus of whatever nature. The occlusion of the esophagus may be very short, or may exist in the form of a fibrous cord which is either quite evident or almost indistinguishable, and which may or may not contain distinct evidences of muscular fibre. The esophagus throughout its extent is generally firmly adherent to the trachea, and the obliterated portion especially so, even almost indistinguishable from the posterior tracheal wall. The obliteration is almost always situated at or a little above the bifurcation of the trachea. When it is of very short length the two fragments of the esophagus frequently appear to overlap each other. The upper fragment of the esophagus

or

is generally dilated, with its walls hypertrophied. It is usually rather funnel-form than cylindrical, and ends blindly at the point of obstruction. As a rule, the lower fragment is of normal calibre for some distance above the stomach, and then gradually tapers in an upward direction, ending in a fistula into the trachea. It is noticeable that the fistula is always in the lower fragment. The only exception recorded is that of Fischer, in which, in addition. to the usual fistula in the lower portion, there was also a very minute opening into the trachea from the upper fragment of the esophagus. The fistula is usually slit-like in form and enters the trachea oftenest shortly above the bifurcation; less often just at the position of the bifurcation. Fistula into the bronchus is extremely rare. Mackenzie found it recorded but 3 times and Happich adds none to the list. We have found I additional case reported by Kirmisson in 1904.

The case which we desire to place on record belongs to the class of obliteration with tracheoesophageal fistula. The notes are as follows:

Joseph Dorland, nine days old, was admitted to the Hospital of the University of Pennsylvania, December 7, 1907. The parents were living and well. Five other healthy children had been born. The patient was a full term infant, delivered with forceps. after a somewhat difficult labor. Since birth he had never been able to swallow. After taking the breast for a moment he would become cyanotic, struggle as though strangling, and then eject milk and usually a large amount of mucus from the nose and mouth. Apparently no nourishment whatever passed into the stomach. There was no vomiting except after nursing. Very small bowel movements occurred about twice a day. There had been jaundice since birth. Examination on admission showed an extremely emaciated child. Attempts to feed it were at once followed by the regurgitation and strangling described. The child died a few hours after admission.

The autopsy revealed the body of a well-formed, emaciated infant. The features of interest in the examination of the internal organs were limited to the esophagus and the adjacent parts. The pharyngeal portion of the esophagus was slightly dilated, but otherwise normal to 4 cm. below the upper level of the larynx, where it terminated abruptly. The inferior portion was normal from the stomach upward to within a short distance of the termination of the upper fragment, where the lumen became narrower and, inclining slightly anteriorly, opened by a slit-like orifice into the trachea slightly above the level of the lower end of the

upper fragment. The accompanying drawing (Fig. 1) gives a diagrammatic representation of the condition which the esophagus exhibited. An examination of the rest of the thoracic and abdominal viscera revealed no additional malformations or other pathological features.

Pathology.-Malformations of the esophagus doubtless arise in various ways, but in no form is the pathological explanation very certain and in some very unsatisfactory:—

Entire absence of the esoph

agus can occasion no surprise, as it is seen only in conjunction with other so extensive and serious malformations.

The occurrence of the complete absence of only a portion of the esophagus is very problematical. As already stated, some traces of esophageal tissue probably always remain. No satisfactory explanation for it seems to offer. Kleb's theory is that in the development of the respiratory organs from the gut-tract too much tissue was consumed at the expense of the esophagus, this resulting in a localized complete disappearance of the tissues of the latter. The cases in which more or less of the esophageal wall is evidently present in the

FIG. 1.

form of a cord or band Kraus explains as resulting from a form of pressure-atrophy. He has observed 3 instances of this anomaly, in all of which there was compression of the obliterated area by an anomalous vessel. In 2 of them this was the right subclavian artery, which arose as an independent vessel and as the last branch from the arch of the aorta, which lay behind the esophagus.

Simple fistula without other lesion is apparently the result of the persistence of the embryological communication between the primitive gut-tract and the cervical portion of the air passages. As this embryonic communication exists between the air passages at or just above the bifurcation of the trachea and the correspond

ing portion of the esophagus, the resulting fistula is practically always at this point.

Congenital stenotic conditions probably depend upon accidental changes in some part of the esophageal wall.

The greatest interest attaches to the pathology of the most common anomaly, i.e., obliteration of the esophagus with tracheoesophageal fistula, and the questions naturally arise, Why fistula is so usually combined with obliteration? Why the obliteration is always close to the position of the bifurcation of the trachea? and Why the fistula is always found in the lower fragment of the in

Illustrating production of congenital obliteration of esophagus with formation of tracheoesophageal fistula. After Shattock.

terrupted esophagus? The most satisfactory explanation of the mode of formation of this abnormality is that proposed by Shattock. According to his view, at the time when the pouch from which the lower air passages develop is formed from the anterior wall of the stomadeum, the posterior wall occasionally participates in the process sufficiently to be drawn forward, thus narrowing the lumen. When, subsequently, the lateral pouching for the formation of the larynx develops from the stomadeum just above this region, so much of the already narrowed lumen is consumed that the communication between the upper and lower portions of the esophagus is cut off and the lower fragment is left communicating with the air passages.

The process can be probably better understood by a glance at the accompanying diagrammatic illustrations taken from Shattock's article. Fig. 2 represents the normal development of the lower air passages from the diverticulum on the anterior wall of the stomadeum, and Fig. 3 the development, from its pharyngeal extremity, of the larynx and upper part of the trachea. In Fig. 4 the abnormal kink in the posterior wall is seen opposite the diverticulum, and Fig. 5 illustrates the subsequent development of the larynx and upper part of the trachea dividing the esophagus into two portions, the lower of which remains in communication with the air passages.

Symptoms. The symptoms of stenosis seen in infancy are similar to those observed later in life. The same is true of dilatation, whether congenital or following stenosis. We shall confine ourselves, therefore, to the symptomatology of congenital obliteration of the esophagus. The children with this condition may be well developed, but are often the subjects of congenital asthenia. Other malformations are very frequently present. From the time of birth there seems to be an unusual amount of mucus constantly expelled from the mouth. This is due to the fact that the oral secretion cannot pass into the stomach as it does in the case of healthy children. No food whatever can enter the stomach, and efforts at swallowing are attended by a prompt regurgitation through the mouth and nose of the food mixed with mucus. There are produced in this way violent suffocative attacks with severe cyanosis. Attempts at passing a catheter into the esophagus meet with the obstruction. Death takes place generally in the first week, exceptionally later, and depends in part upon the constitutional asthenia, in part upon the lack of food, and perhaps still more, as Happich points out, upon the drying of the tissues through lack of water.

Treatment. This is entirely discouraging. Cases of stenosis have recovered, but all instances of complete obstruction have died. The weakness of the child and its early age make operative interference a questionable procedure. Yet gastrostomy offers the only hope. This operation was first done in the case of Steel, and has been performed since then in those of Hoffmann, Happich, Villemin, Kirmisson, and Dickie. The child should be kept on its side to allow the mucus to flow from the mouth. It may be fed through the gastric fistula. Should it recover, an effort may later be made to repair the esophagus by a lateral operation in the neck. This indeed was attempted unsuccessfully in Hoffmann's case as a primary operation, and gastrostomy resorted to later.

(Discussion on page 99.)