CONGENITAL TRACHEOESOPHAGEAL FISTULA F. P.
AND E. I. DOBOS, N[.D.
fistula is one of the many interesting T RACHEOESOPItAGEAL congenital anomalies occurring in the newborn infant. Ordinarily it is found associated with cases of congenital atresia of the esophagus but occasionally may occur in infants who show little, if any, esophageal obstruction. In the literature, there is very little reference to this latter type of tracheoesophageaI fistula. Cautleys in 1917 found only six cases of tracheoesophageal fistula unassociated with other anomalies and forty-five cases of congenital atresia of the esophagus with a fistula between the esophagus and the trachea or bronchi (88 per cent). Strong and Cummins2~ in their article published in 1934 state: " I n reviewing the literature of congenital atresia of the esophagus, almost every author begins with Sir Morrill Mackenzie's collection of sixty-two instances compiled in 1884 for the report of his own single case. According to Mackenzie, the first reported case was that of Durston in 1670." Plass~~ in his presentation in 1919 quotes a description of a ease observed by Thomas Gibson in 1696. EMBRYOLOGY
Atresia of the esophagus apparently may appear very early in embryonic life since Plass 1~ refers to a specimen in the Harvard Embryological Museum in which the abnormality occurs in a human embryo only 18.1 ram. long; Gruenwald~2 has described the condition occurring in an embryo only 9 ram. long, and Ysander, cited by Rosenthal, ~9 in an embryo 8 ram. long. While there is considerable divergence of opinions regarding the cause of congenital tracheoesophageal communications, it is generally conceded that both trachea and esophagus develop from the same embryonal anlage which consists of an entodermal pouch situated in the thorax behind the heart. About the time when the embryo reaches a length of 4 ram., two lateral indentations or grooves appear on either side of the entodermal sac which become gradually deeper and, when they meet in the center, form the septum, dividing the entodermal sac into an anterior tube which belongs to the respiratory system and the posterior tube which belongs to the alimentary system. The completion of the h e a d a t the m e e t i n g of the A m e r i c a n :Fediatric Society, M a y 22-24, 1941, H o t Springs, Va. :From the D e p a r t m e n t of P e d i a t r i c s , Universit:y" of Colorado 1E~edical School, a n d t he Children's I-Iospltal, Denver. 644
GENGENBACH AND DOBOS:
CONGENITAL TRACHEOESOPHAGEAL FISTULA
septum takes place first in the caudal end of the pouch and it extends subsequently upward, in cranial direction. When the embryo is 11 mm. in length, the septum formation is completed. Since fistulous communications between trachea and esophagus represent incomplete septal development and since these defects occur close to the caudal end of the sac, such defects may be recognized long before the septal development is completed in the cranial direction. The location of traeheoesophageal fistulas shows very little variation. In the newborn infant, they are situated at the level of the bifurcation of the trachea or in a distance of 1/~ cm, above or below that point. Consequently, theories that attribute such lesions to trauma or inflammatory changes in fetal life (Luschka, T M Klebs 19b) seem entirely untenable since any portion of the septum should be equally vulnerable to such forces. Similar objections could be raised against the theories attributing the lesions to idiopathic septal deviation (Ribbert, s6 Konopacki, ~7 and Hoffmann'S). Kreuter's ~b theory claiming that atresias of the esophagus may be explained on the basis that the originally solid esophageal anlage fails to develop a lumen may be entirely disregarded as there is no reason to assume that the entodermal anlage is solid at any stage of embryonal development and this theory would leave unexplained the developmental process of tracheoesophageal fistulas which exist without atresia. Probably the most plausible theory is the one advanced by Zausch,~3 attributing the lesion to pressure exerted on t h e primitive entodermal sac by the heart anlage, which acts as a wedge, with the maximum point of pressure directed against one certain anatomical region which coincides with the point where tracheoesophageal fistulas do occur. (According to this concept, if the pressure~is exerted on points higher up, it may interfere with the closure of the palatal clefts.) Rosentha], ~9 however, points out in his excellent review of tracheoesophageal fistulas that there are two valid objections to the acceptance of Zausch's theory, namely: (1) There is no such great proximity between the heart anlage and tracheoesophageal an]age as this theory would suggest since adequate space between the two are clearly demonstrable; (2) should this pressure phenomenon be the prevailing factor, one should expect greater implication of the trachea, which is nearer the point of the wedge, than of the esophagus, and that, of course, is not the ease. TYPES OF ESOPHAGEAL ATRESIA
Vogt 17 has suggested the following classification of the variations of congenital atresia of the esophagus : 1. Complete absence of the esophagus. This type is rare. TM 2. Cases in which there is an upper and a lower segment, each ending in a blind pouch.
OF P E D I A T R I C S
3. Cases with fistulas communicating between the esophageal segments and the trachea or bronchi: (a) With fistula between the u p p e r segment and trachea; (b) with fistula between the lower segment and t r a c h e a or bronchus; (c) with fistulas between both the segments and the air passages. In L a n m a n ' s 33 review of thirty-two cases of congenital atresia of the esophagus occurring in the Children's Hospital of Boston, he reports that there were 19 boys and 13 girls, and of these 32 cases, there were 29 eases, or 91 per cent, of type 3 (b) ; one case of type 2; one case of type 3 (a) and one case of type 3 (c) with some narrowing but not complete obstruction of the esophagus. similar preponderance of cases of type 3 (b) is reported by some of the other observers. Thus Mackenzie 1, 2 in a review o~ 62 cases, reported 40 cases, or 60 per cent, of type 3 (b) ; Hacker and Lotheissen 16 reviewing 223 cases, reported 173 cases, or 77 per cent; and Rosentha119 in reviewing 255 cases, reported 205 cases, or 80 per cent of type 3 (b). Freudenberger and K e r b y 34 have r e c e n t l y reported three cases of congenital atresia of the esophagus with tracheoesophageal fistula, and in one of them, interesthlgly enough, they were permitted after death to introduce a barium suspension into the u p p e r segment of the esophagus and through an opening in the stomach into the lower segment, and then take an x-ray picture which shows both segments of the esophagus, the stomach, and the tracheoesophageal fistula splendidly portrayed. This case was type 3 (b). m a n y authors have written on the subject of congenital atresia of the esophagus (see references) and m a n y cases have been reported , probably 350 cases or more, 35 but unquestionably many cases with and without associated tracheoesophageal fistula have been undiagnosed both during life and at incomplete autopsies, or, as too frequently occurs, because no autopsy is permitted. Plassl~ states that no two definite eases have been reported in the same family. However, G r i e v e and McDermott 31 since then have reported congenital atresia of the esophagus with tracheoesophageal fistula occurring in two successive male births in the same family. These children were born in 1937 and 1938 a n d were both autopsied. The second infant also had an imperforate anus. SYMPTOMATOLOGu A f t e r seeing a ease of congenital atresia of the esophagus with its characteristic symptoms, o n e should have little difficulty in making a diagnosis, or at least suspecting the presence of the condition in subsequent eases. Because of the accumulation of mucus, saliva, and other secretions in the u p p e r segment of the esophagus which tend to spill over into the throat and from thence into the larynx and up into the nasopharynx, the infant shortly after birth has attacks of choking, coughing, and cyanosis.
G E N G E N B A C H A N D DODOS:
C O N G E N I T A L T R A C H E O E S O P H A G E A L :FISTULA
He is unable to nurse when put to the breast, and if water or a feeding is given, the liquid is almost immediately regurgitated from the mouth and through the nose with an exacerbation of the above described symptoms. Between these attacks there is an almost continuous drooling of f r o t h y mucus and saliva from the mouth and f r o t h y mucus from the openings of the nostrils. I f a very small catheter is inserted through the nose or the mouth, it soon encounters a n obstruction in the esophagus. If, in addition to the above symptoms and signs, the u p p e r portion of the abdomen occupied by the stomach becomes distended, one should suspect, as suggested by Brennemann 7' ' that a tracheoesophageal fistula is also present, permitting the entrance of air and secretions into the stomach. DIAGNOSIS
In the past, the presence of congenital atresia of the esophagus has been confirmed by the injection through a small catheter into the esophagus of a barium suspension, or, more recently, of iodized oil, as contrasting mediums for x-ray diagnosis. Lanman, 3. however, in his recent article makes the following statemerit: " W h e t h e r or not there is a fistula associated with the atresia, the giving of barium sulfate by mouth is accompanied by great danger and should n e v e r be done. Aspiration of barium sulfate alone into the air passages results in an irritative type of pneumonia. Aspiration of iodized poppyseed oil is relatively harmless in itself, but even with the most careful technic there will be some aspiration of the oil plus mucus and saliva containing the oral organisms from the u p p e r segment of the esophagus. It is important also to realize that the use of a contrast medium will not give any helpful diagnostic information that cannot be obtained without its use. Before the plain roentge~ogram is taken, a soft rubber catheter should be passed down the esophagus as far as it will go. The level of the upper pouch can be clearly demonstrated by this procedure. I f there is no gas in the stomach or bowel, the anomaly is either type 2 or type 3 (a). I f the catheter meets an obstruction and there is gas in the stomach or bowel, it is either type 3 (b) or t y p e 3 (c). F o r practical purposes this is all the differentiation needed preoperatively." Another practical diagnostic sign is the lack of lanugo hair in the meconimn, thus indicating some form of obstruction in the u p p e r portion of the alimentary tract. TREATMENT
Treatment of the acute symptoms is purely symptomatic, with the use of aspiration and the administration of oxygen, Alpha-lobe]ine, and perhaps cardiac stimulants, such as coramin, when indicated. Blood transfusions are temporarily helpful, and to combat the dehydration
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which soon occurs, intravenous injections of normal saline, or Ringer's or Hartmann's solutions with the addition of glucose should be given. Feedings to sustain the strength of the infant can be given only per rectum or through a catheter inserted into the stomach after a gastrostomy is done. These measures, especially rectal feeding, are usually poorly tolerated and of no avail in keeping the infant alive very long; in the majority of eases, death occurs during the first week of life. However, Plass, 1~ Phelps, is Rosenthal and Himmelstein,21 and Trump 22 report cases where the infant has survived for from fourteen to thirty-seven days. Surgery in the first few days of life alone offers hope for the ultimate survival of the infant. Many types of operation have been described in the literature, but all, so far, usually have been unsuccessful. The ideal operation apparently should be one in which the upper and lower segments of the esophagus can be joined together and the opening kept patent until healing occurs. At the same time, the traeheoesophagea] fistula should be tied off at its tracheal end to pTevent the regurgitation of some o~ the feeding up through the lower segment of the esophagus and the fistula into the trachea, causing an aspiration pneumonia. If the infant does not die almost immediately or in the first few days of life, death in atresia of the esophagus is usually due to a bronchopneumonia caused by a spilling over of the accumulated secretions in the upper segment of the esophagus into the larynx, thus provoking an aspiration pneumonia. In the comparatively few cases of tracheoesophageal fistula without esophageal atresia, the treatment in the acute attack again must be symptomatic with the addition of chemotherapy to combat the recurring attacks of bronchopneumonia caused by the regurgitation of some of the feedings into the air passages. Even in these cases, however, it would seem desirable that a successful operation be devised to tie off the tracheoesophageal fistula at its juncture with the trachea to prevent possible regurgitation of the feeding through the fistula. CASE REPORTS
Colorado General Hospital CASE ] . - - B a b y boy S. was admitted to Colorado General Hospital on the service of Dr. L. C. Wollenweber, July 1, 1936, at the age oi 3 days because of vomiting of all feedings. X-ray examination with barium was made on July 3 by Dr. E. A. Schmidt who reported complete atresia Of the esophagus at the level of the fourth thoraci~ vertebra. The consulting surgeon, Dr. J. 1~. Foster, Jr., advised against operation as the condition of the i n f a n t would not warrant such radical surgery. The patient died on July 7 at the age of 9 days. Autopsy performed by Dr. Anderson revealed congenital atresia of the esophagus ending in a blind pouch 8 cm. above the cardiac end. J u s t below the atresia, which extended 4 ram., there was a communication with the trachea about 2 ram. in size. Death was due to a bilateral bronchopneumonia.
GENGENBACH AND DOBOS:
CONGENITAL TRACHEOESOPHAGEAL FISTULA
CASE 2 . - - B a b y girl J. was born in Colorado General Hospital on Dec. ]9, 1937, and immediately t r a n s f e r r d to the pediatric department, service of Dr. E. Friedman, because of marked respiratory difficulty, excess of mucus in nose and throat, and cyanosis. X-ray examination with barium on Dee. 21 by Dr. E. A. Schmidt revealed " w i d e n i n g of the upper third of the esophagus combined with complete obstruction suggests congenital atresia. '~ The patient died on Dec. 2 2 a t the age of 3 days. Autopsy by Dr. Benner revealed normal esophagus for about 4 era., ending in a blind pouch and attached to the lower segment of the esophagus by a cord. There was a tracheoesophageal fistula extending from just above the bifurcation of the trachea to t h e lower s e g m e n t ' o f the esophagus. There also was present a congenital atresia of the duodenum and a bronchopneumonia. CASE 8 . - - B a b y girl B. was admitted to the service of Dr. R. P. Forbes and Dr. R. J. McDonald, Jr., from the obstetrical service on May ]3, 1939, the day of r birth~ because of difficulty in b r e a t h i n g and cyanosls. X-ray examination with barium on May 13, by Dr. McMullen revealed " A p p a r e n t atresia of the esophagus ending at the fourth thoracic vertebra. Marked gas accumulation in the gastrointestinal t r a c t . " The patient died the same day. Autopsy by Dr. Boughton: " U p p e r portion of esophagus ends in a blind pouch 4.5 cm. below the larynx. At bhis point, there is a fibrous cord attachment to t h e trachea and also to the lower segment of the esophagus. A traeheoesophageal fistula joins the trachea and the lower segznent of the esophagus. There was also an aplasia of the left kidney which weighed 4.62 Gm. Cause of death, bronchopneumonia. ' ' The Children's Hospital CASV~ 1.--W. It. F., a male 5 days old, was admitted May 26, 1928, on the service of Dr. G. M. Bliekensderfer. His weight at b i r t h was 7 pounds, 2 ounces; weight at the time of admission,-6 pounds, 12 ounces. There was immediate vomiting of all fluids, accompanied b y eyanosis; passed meconinm but no normal fecal m a t t e r ; was dehydrated. Laboratory Report. Urine: Albumin + + + ; acetone, ++. Blood: Hemoglobin, 110 ( D a r e ) ; red blood cells, 7,100,000; white blood cells, 9,800; polymorphonuclears, 54; lymphocytes, 44; large mononuclears , 2 per cent; coagulation time, three minutes. On May 26 an x-ray examination by Dr. F. B. Stephenso~i showed inserted catheter and ingested barium both arrested at the level of the fourth dorsal vertebra; no barium in the stomach. Esophagoscopy by Dr. T. E. Carmody on the same day revealed a congenital atresia of the esophagus at the junction of the u p p e r and middle thirds. On May 27 a Wetzel gastrostomy by Dr. G. B. Packard, Jr., revealed small stomach; palpating finger unable to feel lower end of esophagoscope. P a t i e n t died on May 28 on the eighth day of life. Autopsy by Dr. P. Hilkowitz and Dr. C. B. Kingery revealed the upper segment of the esophagus terminated in a blind sac, which measured 6 cm. in length and 1 cm. in diameter. The lower segment of the esophagus, which represented an entirely separate unit from the upper segment, was smaller than usual; the caliber o f the lumen was diminished. The consistency of the esophageal wall in the lower segment was firmer t h a n usual, and the upper end of the lower segment entered the trachea j u s t above the bifurcation; several wedge-shaped hemorrhagic infarcts were in the left lung. Diagnosis was tracheoesophageal fistula, with esophageal atresia and hemorrhagic infarcts of the lung. CASE 2.--B. J. g., a girl, 3 months old, was admitted Feb. 11, 1934, on the service of Dr. F. P. Gengenbach. I n f a n t was born at the eighth month of pregnancy.
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Mother had albuminuria. Birth weight~ 5 pounds 8 ounces; weight at the time of admission, 5 pounds, 9 ounces. There was a history of repeated respiratory difficulties with cyanotic attacks since birth. The i n f a n t was unable to nurse and regurgitation of all feeding was accompanied by eyanosis. Laboratory ~indings. Urine: Negative except trace of albumin. Blood: Hemoglobin, 78 (Dare); red blood cells, 3,500,000; white blood cells, 13,000; polymorphonuclears, 42; lymphocytes, 52; large mononuelears, 6 per cent. An x-ray examination by Dr. K. D. Allen on Feb. 12 is reported as follows: " T h e r e is definite intensification of trunk and hilum shadows on the right~ suggesting a low-grade type of infectious process or passive congestion resulting from inet~cient eardia. 'No shifting of the h e a r t . " On Feb. 13, while the child was being fed, she suddenly developed marked respiratory di~culty, became very cyanotic, and llmp, An x-ray examination by Dr. K. D. Allen on Feb. 14 is reported as follows: ~Ingestion of barium reveals lateral deviation of the esophagus to the left and trachea to the right. Although the bariurL, passes freely into the stomach, some swallows of barium, or portions thereof, enter the trachea above the fourth dorsal vertebra, and it passes into both muln stem bronchi and some smaller divisions. The x-ray signs indicate some faulty deglutition mechanism of the epiglottis." After the ingestion of the barium mixture, the patient developed a severe bronchopneumonia and died on Feb. 14 on the fourth day after admission. On Feb. 16 autopsy was performed by Dr. E. I. Dobos. Exploration of the esophagus disclosed no atresia or any alteration in the contour of the lumen of the esophagus. Upon the opening of the lumen buried in the long~itudinul folds of the esophageal mueosa, a very small umbilication was noted. Upon the spreading of the folds of the mucosa, a small fistulous tract appeared, measuring 4 ram. in diameter. This opening is situated on the anterior wall of the esophagus, and, upon the insertion of the probe, it was found to establish communication between the lumen of the esophagus and the lumen of the trachea. The course of this communicating opening is directed upward and anteriorly from the esophagus in an angle of approximately 30 ~. The opening of the fistula is situated on the posterior wall of the trachea 3 mm. above the bifurcation of the trachea. The lumen of the esophagus was empty. I n the lumen of the trachea and in the large bronchi a large amount of milk curds were encountered. Disseminated bronchopneumonic foci were found in the right upper and middle lobes. The heart was greatly enlarged which was due chiefly to the enlargement of the right ventricle. The right ventrieular myocardium was greatly hypertrophied; no valvular lesions or any defect in septal development were found. However, there was a wide patent ductus arteriosus. The diminution of the diameter of the aorta in the arch suggested a possible coarctation. On the exploration of the abdominal cavity, no kidney was found on the right side. On the left side there was a very large fused kidney situated just above the level of the promontory, and it presented a very large, disk-shaped organ supplied by two repal arteries, thus indicating that the structure represented fusion of both right and left kidneys. Two separate ureters and pelves were present, the ureters entering the bladder in the usual loeatio~. Diagnosis was tracheoesophageal fistula without atresia of the esophagus, acute bronehopneumoni% patent ductus arteriosus, absence of the right kidney, and fusion of two kidneys on the l e f t side. CASE 3.--A. M. W., a girl, 4 days old, was admitted Nov. 7, 1935, on the service of Dr. 1%. P. Forbes. She was not weighed at birth; weight at time of admission, 3 pounds, 14 ounces. Since birth the i n f a n t had choked on mucus and became cyanotic every time feeding was attempted.
GENGE~TBACH AND DOBOS: CONGENITAL TRACHEOESOPHAGEAL FISTULA
Laboratory Findings. Urine : Albumin, + + +. Blood : I-Iemoglobin, 10O (Dare) ; red blood cells, 4,850,000; white blood cells, 1O~000; polymorphonuclears, 61; lyrephoeytes, 37; large mononuclears, 2 per cent; coagulation time, three minutes. On Nov. 8 an x-ray examination by Dr. J. S. Bouslog showed an obstruction of the esophagus at the level of the second dorsal vertebra. On Nov. 11, 1935, gastrostomy was performed by Dr. G. B. Packard, Jr., and catheter inserted for feeding. Patient died on :Nov. 15 on the thirteenth day of life. Autopsy was performed by Dr. E. I. Dobos and is reported as follows: " T h e r e are small bronehopneumonic foci, in both lungs. The lower end of the upper segment of the esophagus terrrlinated in a blind sac. The upper segment measures 8 era. in length. The upper end of the lower seg~nent terminates in the trachea and enters the tracheal wall just above the bifurcation. Upon the examination of the large vessels~ a patent ductus arteriosus was found. Diagnosis was traeheoesophageal fistula~ with esophageal atresia, and bronchopneumonia. CASE 4.--L. J. It., gir]~ ] day old, was admitted May 14, 1937, on the service of Dr. E. S. Pratt. Weight, 3 pounds 15 ounces; had had several cyanotic attacks since birth. Laboratory Report. Urine: Speeime~n not obtained. Blood: Hemoglobin, 87 (Dare); red blood cells, 4,590,000; white blood cells, 16,600; polymorphonuelears, 70; lymphocytes, 27; large mouonuelears, 3 per cent. Coagulation time, three minutes; two nucleated red cells per 100 white blood cells. On May 15~ an x-ray examination by Dr. F. B. Stephenson showed small catheter i~ esophagus passes t~ the level of the third dorsal vertebra where it deviates to the right. Barium mixture collected at same level at the end of the catheter. No barium passed into the lower esophagus or into the trachea. On May 16 the patient died on the fourth day of life. Autopsy was performed by Dr. Dobos: ~'There is an esophageal atresia, the upper segment of the esophagus terminating in a blind sac measuring 8 cm. in length. The upper end of the lower segment of the esophagus terminates in the posterior wail of the trachea in form of a traeheoesophageal fistula. The diameter of the fistulous tract is 8 ram. No other anomalies were f o u n d . " Diagnosis was traeheoesophageal fistula with atresia of the esophagus. CASE 5.--J. L. E., a 10-month-old white female, was admitted Jan. 27, 1940, on the service of Dr. F. P. Gengenbach with a history of being in St. Joseph~s Hospital for seven months following delivery where she had been under the care of Dr. :F. Craig Johnson, a former associate. Her color was poor when b o r n ; three days after delivery, she began having attacks of eyanosis and coughing when being fed. The chest which would be clear to percussion before feeding became full of rules after feeding. Bronehoseopy and iodized oil injections were carried out by Dr. C. H. Darrow. The oil was seen in the chest by x-rays. History revealed that this child had had many attacks of pneumonitis. She had done fairly well on a farina formula by garage and had been able to take feedings from a bottle during last few weeks before admission to the Children's Hospital. Physical examination at time of admission revealed a moderately ill child, quite irritable, 10 to 12 months of age~ and small in stature. There were many rhonehi and moist rMes in the chest transmitted bilaterally; no definite localization. Laboratory Findings. Blood: On admission it showed 73 per cent hemoglobin; red blood cells 4,370~000; white blood cells, 15,200; polymorphonuclears, 35 per cent, lymphocytes, 65 per cent. Urine: Negative except for 1§ acetone. Throat culture showed hemolytic streptococci and staphylococci. On subsequent admissions with upper respiratory infections~ the white blood cells ranged up to 29,000.
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The child was put on sulfanilamide, which she tolerated well; the concentration on Jan. 30 was 6 rag., and on Feb. 1 was 5 rag. Temperature on admission was 103.4 ~ F. and fell gradually, only to become elevated again on Feb. 1 to 102~ then it fell to normal and remained practically at normal until the time of discharge. X-rays were taken of the chest on Jan. 31 which were read b y Dr. F. B. Stephenson with the following findings: ' ' There is an area of density in the right upper lobe, adjacent to the mediastinum and also some cloudiness around the right hilum, attributable to pneumonitis. The thymus is not unduly large. Since the upper mediastinal shadow and the trachea deviate to the affected side, some partial obstruction of the bronchial system is suspected." 0 a Feb. 13 the child was discharged, seventeen days a f t e r admission. Final diagnosis was deferred; condition improved. She was readmitted on Feb. 25 with another upper respiratory infection, discharged April 21 and taken to Children's Memorial Hospital, Chica~o, for consultation with Dr. Joseph Brennemann. She had similar respiratory attacks there and later was esophagoscoped and bronchoscoped by Dr. Paul Itolinger. His report follows : "On attempting to introduce an esophagoscope, a blast of air and frothy saliva was expelled into the pharynx from behind the larynx with each cough. Inspection of the larynx revealed the airway patent, b u t the left cord was thickened and did not move with respiration. I t appeared to be fixed in the midline. "Bronchoscopy: The trachea contained a large quantity of frothy liquid which had the consistency of saliva. A f t e r both bronchi were inspected and found to be normal, the trachea was re-examined. The frothy, clear fluid described above appeared to have its origin in a small orifice slightly to the left of the midline, 2 to 3 era. above the bifurcation of the trachea. This measurement needs verification. ~Re-examination of the esophagus with the i n f a n t esophagoscope: An apparent esophageal obstruction was found 19 cm. from the upper teeth. Time did not permit satisfactory examination and identification of pathology at this p o i n t . " Readmitted to Children's Hospital May 29; discharged July 20. l~eadmitted July 22; discharged Sept. 29. Readmitted Oct. 21 ; discharged Nov. 11. l%eadmitted Dec. 12; discharged Jan. 8, 1941. Readmitted April 25; discharged May 1. Present age, 2 years; weight, 28 pounds. CASE 6.--8. L. B., a 7-day-old girl, was admitted Feb. 18, 1940, on the service of Dr. F. P. Gengenbach with a history of vomiting since birth. I n f a n t had been fed by hypodermoclysis since birth. Each feeding attempted by mouth w~s vomited. Physical examination revealed a poorly nourished baby with a rather weak cry; skin was dry and icteric in color; fontanels were open; posterior pharynx appeared injected; lungs clear to auscultation and percussion; heart, negative; umbilical cord in good condition; abdomen, negative. Provisional diagnosis was congenital atresia of esophagus and bronchopneumonia. Laboratory Findings. Urine: Essentially negative. Blood: On Feb. 18, hemoglobin, 95 per cent; red blood cells, 5~080,000; white blood cells, 10,100; polymorlohonuelears, 61 per cent; lymphocytes, 39 per cent; on Feb. 26, hemoglobin, 75 per cent; white blood cells, 10,800; polymorphonuclears, 51 per cent ; eosinophiles, 4 per cent; and lymphocytes, 45 per cent. X-ray Examination, Feb. 18. Esophagus: Barium mixture swallowed by patient remained persistently in a pouchlike dilatation in the esophagnls at the level of the bifurcation of the trachea. Over a period of six hours none passed into the stomach. A catheter inserted into the esophagus did not pass beyond t h a t point. Conclusions: Atresia of the esophagus a t the level of the fourth thoracic interspace. Examination and report by Dr. F. B. Stephenson.
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CONGENITAL TRACHEOESOPHAGEAL FISTULA
Feb. 19, Supplementary Report: There was gas present in both the stomach and small intestine. Feb. 23, Trachea: After instillation of lipiodol into the trachea by Dr. H. I. Laff~ films i n the horizontal, semierect~ and Trendelenberg positions failed to show that any of the lipiodol had entered the esophagus nor was there any in the s~omaeh. Feb. 24, Supplementary :Examination: The anteroposterior view showed persistence of lil)iodol in the right upper lobe with occlusion of some of the bronchial branches. Chest 24 hours later. The r i g h t upper lobe was cloudy and there was lipiodol in the bronchial branches in t h a t lobe. This baby was seen in consultation by Dr. G. B. Packard, Jr., who advised against any surgery. Child died March 10 on the twenty-eighth day of life. Autopsy by Dr. E. L Dobos whose findings were as follows: 'CThe esophagus ends in a blind pouch. The length of the upper segment of the esophagus is 2 cm. The lumen is considerably dilated and the wall is greatly hypertrophied. The lower segment of the esophagus was explored from the cardiac end, the lumen was found patent, and upon probing the lumen of the esophagus~ it was found t h a t the upper end of the lower segment terminates in the posterior wall of the trachea by a fistulous t r a c t which perforates the posterior wall of the trachea in a n acute angle, the fistulous t r a c t running upward arid anteriorly. There was a small fibrous cord connecting the lower end of the upper segment with the upper end of the lower segment of the esophagus, bringing about a complete atresia of the esophageal lumen. The diameter of the tracheoesophageM fistula is approximately 5 r a m . ' ' I t is interesting to note t h a t Brennemann~ in the concluding sentence of his presentation in 1913 said, ' ' W h i I e the u t t e r hopelessness of these cases, i f untreated, justifies surgical procedures t h a t would otherwise seem too daring; nevertheless the physician who, a f t e r making his diagnosis of congenital atresia of the esophagus, decided to let his little patient die undisturbed, as in Case 3 ( B ' s ) , can amply justify his course." CASE 7.--J. A. S,, a 21~-month-old white female, was admitted Oct. 8, 1940, on the service of Dr. R. P. Forbes. P a t i e n t was a full-term normal infant, w e i g ~ n g 8 pounds, 4 ounces at birth. For several days a f t e r b i r t h patient took her feedings quite well; thereafter, she began to have choking and cyanotic spells coincidental with each feeding; this persisted until time of admission. She had had some diarrhea and vomiting simultaneous with formula changes. The day before, on routine weekly check-up, the doctor found the patient to have an elevated temperature and patient had some congestion in her lungs. She began to have a cough when she was 3 weeks old and this persisted until admission. Cough more severe during twenty-four hours previous to admission. Up to t h a t time she took feedings quite well. Physical examination revealed marked purulent discharge from nose. P a t i e n t had rapid grunting respiration with suprasternM tug. I n s p i r a t i o n and expiration of equal intensity; many dry rMes in r i g h t base posteriorly and left apex posteriorly. Provisional diagnosis was (1) bronchopneumonia, (2) possible tracheoesophageaI fistula. Laboratory Findings. Urine: Within normal limits. Blood: Count on Oct. 9 showed 85 per cent hemoglobin, 4,200,000 red blood cells, 21,400 white b l o o d cells, 21 per cent polymorphonuclears, 76 per cent lymphocytes, and 3 per cent large mononuclears. On Oct. 12 hemoglobin was 75 per cent with 17,100 white blood cells and 28 per cent polymorphonuclears. On Oct. 10 sulfathiazole determination was r rag. X-ray Findings (Oct. 8). Chest: Examination by Dr. J. S. Bouslog showed congestion throughout both lungs and this was more marked on the right, particularly in
THE JOURNAL OE PEDIATRICS
the right upper lobe, indicating a bronehopneumonia. On Oct. 11 a picture was made after lipiodol injection into the esophagus by Dr. I~. I. Laff. The lipiodol was seeI~ in the left main bronchus and in the trachea. ~ o definite evidence o2 a fistula. Admission temperature was 100~ admission weight, 9 pounds, 9 ounces. P a t i e n t started on sulfathiazole. Respirations on admission~ 104 per minute. During hospital stay the chest did not clear entirely; patient coughed with feedings. The i n f a n t was discharged Oct. ]5. She was readmitted Dec. 3 with a similar history and type o2 infection, treated with sulfathiazole, and discharged Jan. 21. Subsequently the patient was admitted to the Colorado General Hospital with a similar attack from which she again recovered under the same treatment. Esophagoscopy and bronchoscopy so f a r have not been permitted. This child is now about 8 months old. SUMMARY
In this study of 3 eases observed at the Colorado General Hospital and 7 eases observed at the Children's Hospital, 8 cases of congenital tracheoesophageal fistula proved at autopsy, 1 case proved by esophagoscopy and bronchoseopy, and 1 case suspected clinically, but not yet proved, are reported. Seven of t h e nine infants with proved cases, or about 80 per cent, had a congenital atresia of the esophagus; the other 3 infants, including the I suspected clinically, had little, if any, esophageal obstruction and could take their feedings from a bottle. The 9 proved cases were Type 3 (b), according to Vogt's classification. Of the 10 eases, only 2 were boys and 8 were girls. Most statistics show about an equal number of each sex. REFERENCES 1. Mackenzie, M.: Arch. Laryngol. 1: 301, 1880. 2. Mackelmie, M. : ~_ Manual of Disease of the Throat and Nose, New York, ]884, William Wood & Co., vol. 2, pp. 149-158. 3. (u) Schridde, H.: Virchows Arch f. path. Anat. 191: 178, 1908. (b) Kreuter : Cited by Schridde.3 4: Lateiner, N.: Wien. klin. Wehnsehr. 2:2: 53, 1909. 5. Griffith, J. P. C., and Lavenson, 1~. S.: Arch. Pediat. 26: 16], ]909. 6. l~iehter, H . M . : Surg. Gynec. & Obst. 17: 397, 1913. 7. Brennemann, J . : Am. J. Dis. Child. 5: 143, 1913. 8 . Cautley, E d m u n d : Brit. ft. Child. Dis. 14: 1, 1917. 9. Brennemann, J.: Am. J. Dis. Child. 16: 143, 1918. 10. ]?lass, E . D . : Johns Hopkins Hosp. Rep. 18: 259, ]919. 11. Peterson, O. I-I.: Beit~. z. klln. Chir. 124: 705, 1921. 12. ]~eynolds, R. P., and Morrison, W . W . : Am. J. Dis. Child. 21: 339, t921. 13. Zausch, P.: Arch. f. path. Anat. 234: 94, 1921. 14. Rienhoff, W. F. : Bull. Johns I-Iopkins IIosp. 25: 271, 1924. 15. Ssubanejew, F r a n k : B i e k h a m ' s Operative Surgery, Philadelphia, 1924, W. B. Saunders Co., vol. 4. 16. }tacker, V., and Lotheissen, G. : ~ e u e Deutsche Chit. 34: 192, ]926. 1~. u :E. C.: Am. J. Roentgenol. 22: 463, 1929. 18. Phelps, K . A.: Ann. Otol., l~tlin. & Laryng. 39: 364, ]930. 19. l~osenthal i 2/. K.: Arch. Path. 12: 756, 1931. (~) Luschka: C i t e d by Rosenthal.z9 (b) Klebs : Cited by Rosenthal.19 (c) Forssner: Cited by Rosenthal.19 (d) Streeter: Cited by Rosenthal.19 ( e ) Ysander, F. : Cited b y l~osentha].19 20~ Kelly, A. B.: J . L~ryng. & Oto]. 46: 521, ]931.
GENGENBACH AND DOBOS: 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32. 33. 34. 35. 36. 37. 38.
CONGENITAL TRACttEOESOPHAGEAL FISTULA
Rosenthal, A. H , and Himmelstein~ U.: Arch. Pediat. 49: 444, 1932. Trump, I ~. A.: J. PEDIAT. 2: 212, 1933. Strong, R. A , and Cummins, H.: Am. J. Dis. Child. 47: 1299, 1934. Leven, N . L . : J. Thoracic Surg. 6: 30j 1936. Gage~ Nims, and Ochsner, Alton: Ann. Surg. 103: 725, 1936. Ochsner, Alton: Surgical Treatment of Esophageal Atresia with TracheoEsophageal Fistula in Brennemann's Practice of Pediatrics, Hagerstown, Md., W. :~. Prior Co., Inc., 1937, vol. 2, chap. 54, p. 2:1. Parmalee, A. H.: Diseases of the Esophagus and S~omach in Brennemann's Practice of Pediatrics, Hagerstown, Md., W. F. Prior Co., Inc., 1937, vol. 3, chap: 5, p. 3. Gamble, H . A . : Ann. Surg. 107: 701, 1938. Imperatori, C. J.: "Arch. Otolaryng. 30: 352, ]939. Shaw, Robert: J. Thoracic Surg. 9: 213, 1939. Grieve, J. G, and McDermott, J. G.: 'Canad. M- A. J. 41: 185~ ]939. Gruenwald, Peter: Anat. Rec. 78: 293, 1940. Lanman, T . H . : Arch. Surg. 41: 1060, 1940. Freudenberger, C. B., and I/:erby, J . P . : l%cky Mountain Mo J. 38: 292, 1941. Oehsner, Alton: Personal communication. Ribbert, Hugo: Lehrbuch d. allgem. Pathol. u. d. pathol. Anat., 1919, ed. 6. Konopacki, M.: Centra]bl. f. allg. Path. u. path. Anat. 23: 368, 1912. Hoffmann, W.: Atresia oesophagi et communicatio inter oesophagum et trachearn, Inaug. Diss., Greifswald, 1899.