CONGENITAL ATRESIA OF THE ESOPHAGUS WITH TRACHEOESOPHAGEAL FISTULA

ALBERT MATHIEU, M.D., F.A.C.S. AND HERBERT E. GOLDSMITH, M.D.

PORTLAND, ORE.

C ONGENITAL atresia of the esoph-

agus is generaIIy considered a rare deveIopmenta1 anomaIy. This paper

presents 2 such cases occurring in the same city, less than two months apart. We are indebted to Dr. S. G. Henricke for permis- sion to use his case (Case II).

Since the first reported case by Durston,’ in 1670, there have been 255 cases as re- ported by RosenthaI in 1931. This report incIuded 8 cases of his own. HistoricaIIy, the case reported by Thomas Gibson3 in 1696, is of interest for the thoroughness of recorded symptomatoIogy, and the post- mortem findings are such that no doubt is Ieft in the reader’s mind as to what deveIopmenta1 pathoIogic condition ex- isted. This case report has been reproduced in detai1 by PIass4 and makes very quaint and interesting reading.

In the EngIish and American Iiterature, exceIIent reviews on this subject have been made by GriIIith and Lavenson” (1909), CautIey; (1917), PI ass (1919), and Rosen- tha1 (1931). PIass reviewed 204 references to congenita1 atresia of the esophagus, 137 of which he was abIe to verify himseIf. The others were either unconfirmed cases or repetitions. His work incIudes 18 pages of bibIiography and covers the period from I 703 to 1916. The work of RosenthaI in 1931 has added 8 new cases. His paper is cIassic from the standpoint of pathology and etiology.

CLASSIFICATION OF ESOPHAGEAL ANOMALIES

BaIlontyne7 in 1904, from a very thorough study of the Iiterature, made the fohowing cIassification of maIformations of the esophagus:

I. Absence of the esophagus in toto 2. Termination of the esophagus in a

simpIe cul-de-sac

3. Termination of the esophagus in a simpIe cuI-de-sac, the Iower end of the cana communicating with the trachea or a bronchus

4. TracheoesophageaI fistuIa, without any other anomaIy of the esophagus itseIf

5. EsophageaI diverticula 6. Membranous obstruction of the

esophagus 7. DoubIe esophagus. About 73 per cent of a11 esophagea1

maIformations are of Type 3 in this cIassi- hcation and the cases presented here faI1 into this group.

ETIOLOGY

The etioIogy of congenita1 atresia of the esophagus is stiI1 somewhat obscure. The background is strictIy theory. The earIy writers heId to the theory that congenita1 atresia of the esophagus resuIted from some intrauterine inffammatory or trau- matic process. We now beIieve that a deveIopmenta1 error is the cause of this defect. This error takes pIace earIy, prob- abIy, before the end of the first month of intrauterine Iife, since in the 4 mm. embryo, the esophagea1 and trachea1 divi- sions are distinct. Kreuter8 feeIs that persistence of the earIy embryona1 stage, wherein the esophagus is a soIid tube, causes the atresia. SchriddIe,g however, finds that in the human embryo, the esophagus has a Iumen at a11 stages of deveIopment. This distinctIv shatters Kreuter’s theory. Keith and SpicerJo be- Iieve that the IateraI tracheoesophagea1 ridges and foIds, which normahy proceed horizontahy backward to meet the pm- monary buds and the esophagus, are diverted and proceed obIiqueIy backward and dorsaiward to produce the atresia.

233

234 American Journal of Surgery Mathieu & Goldsmith-Atresia NOVEMBER, ,933

Zeit” “feeIs that arrested deveIopment of

the IateraI ridges which unite incompIeteIy any fluids. It had frequent attacks of cyanosis

in the midIine, as the IateraI trachea1 and when taking Iiquids by mouth, each one of which had been preceded promptIy by regurgi-

FIG. I. Case I. Anteroposterior roentgenogram taken antemortem. Stomach greatly distended with air. Barium in upper, diIated bIind esophageal sac.

dorsa1 esophagea1 tubes are forming, wouId

expIain the fistuIa but not the atresia.

For both to be caused by one factor, a fauIty anIage with resuIting maIformation must have existed. ” RosenthaI feeIs that the deveIopment of the anomaIy seems to rest on an earIy fundamenta1 change in the endoderma1 ceIIs that are to give rise to the esophagus, and not on any primary concomitant abnormaIities. This change, he thinks, may be genetic and reIated to the anterior end of the neurenteric cana1.

SYMPTOMATOLOGY

The symptomatoIogy of congenita1 atresia of the esophagus is remarkabIy constant. The foIIowing 2 cases are cIassic in showing this constancy.

CASE I. Baby N. was born approximateIy three weeks premature; its birth weight was 2620 gm. It was the first baby of a mother who had a normaI pregnancy and the deIivery of the baby was uneventfu1. The baby was seen by one of us (H. G.) when thirty-six hours oId and unti1 that time the baby had not retained

FIG. 2. Case I. Lateral roentgenogram taken ante- mortem. WideIy diIated stomach fiIIed with air and barium in upper esophagea1 sac.

tation. The baby took water eagerIy by mouth but did not retain it. Water was given by tube and was promptIy regurgitated through the nose. The baby had bowe1 movements of norma meconium without signs of bIood. The baby had voided no urine since birth. An effort to pass a tube into the baby’s stomach reveaIed the fact that there was a definite obstruction at about IO cm. from the Iips.

At the first examination the baby was, to a11 appearances, physicaIIy normaI. The skin was moist, muscle tone was good, there was no evidence of dehydration. The fontaneIIes were neither buIging nor depressed. The eyes were cIear and bright. A smaI1 amount of bIoody, frothy mucus was noted at both externa1 nares. The throat findings were negative. The chest presented no outward evidence of any patho- Iogic condition. Many mucous raIes were heard in the Iungs. There was no aIteration of breath sounds and no duIIness or flatness. The stomach area appeared somewhat distended. The per- cussion note over the stomach was tympanitic and the abdomen was flat and soft. No masses nor areas of tenderness nor evidences of rigidity were present. The bIadder was distended to about 2 cm. above the symphysis. A marked

NEW SERIES VOL. XXII, No. z Mathieu & Goldsmith--Atresia American Journal of Surgery 235

phimosis of the prepuce was present. The returned from the surgery in good condition.

t.esticIes and scrotum were negative. The ex- Two hours Iater the infant was given I ounce

tremities presented no abnormalities except for of a IO per cent glucose solution through the

FIG. 3. Case I. Anteroposterior roentgenogram taken post mortem. Stomach has been fiIled with barium through gastrostomy tube. Note that barium has traversed into lower end of esophagus and by way of smalI fistuIous tract into point of trachea near bifurcation.

the scar on the Ieft hand where a supernumerary thumb had been removed immediateIy after birth.

A catheter was passed into the esophagus and an obstruction was felt about IO cm. from the Iip margin. An attempt to pass water through the catheter produced a severe attack of cough- ing, regurgitation, and cyanosis after about 54 ounce had been introduced. Barium was in- jected into the esophagus and with the aid of the x-ray and fluoroscope, it was seen that the esophagus was fiIIed for part of its length and ended abruptIy in a bIind sac. There was no sign of any barium in the stomach which was fiIIed with a Iarge air bubbIe. (Figs. I and 2.)

During this procedure the infant deveIoped marked asphyxia, but recovered when most of the barium was “milked” out of the mouth and nose.

A gastrostomy was done by Dr. Thomas Joyce at 9:45 P.M., January 23, approximateIy forty-five hours after birth. The stomach was found diIated and fiIIed with air. No ffuid or barium was found in the stomach. The infant

FIG. 4. Case I. LateraI roentgenogram taken post mortem. Note that barium extends from stomach into lower esophagus and into trachea.

gastrostom?- tube. There was an immediate expuIsion of bloody, forthy materia1 through the nose and mouth. The cyanotic attack was extreme, necessitating the use of proIonged artificia1 respiration and oxygen. The infant recovered from this attack onIy to expire thir- teen hours Iater, after a marked eIevation of temperature and repeated attacks of cvanosis, cough and dyspnea. The chest examination previous to death showed a marked, diffuse bronchopneumonia.

The accompanying iIIustrations (Figs. 3 and 4) were taken post mortem before autopsy.

CASE II. Baby E., a femaIe child, was born November 30, 193 I, about six weeks premature. Its birth weight was I 132 gm. Examination of the infant at birth reveaIed no abnormalities. Because of prematurity the baby was pIaced in an incubator, but did not do weI1. It was soon noted that the infant couId not swaIIow and that it was spitting up smaI1 bits of mucus. With the passage of a catheter an esophageal obstruction was noted about 5 cm. from the Iips. A diagnosis of esophagea1 stricture was made at that time. Due to prematurity, no attempts at ?c-ray study were made. The infant died three days after birth.

236 American Journal of Surgery Mathieu & GoIdsmith-Atresia NOVEMBER, ,933

There is a cIassic simiIarity in a11 cases of atresia of the esophagus with trachea1 fistuIa. The symptomatoIogy presents a

I_ .-._. ._i.“_“.~ _- -. .

FIG. 3. Case I. Post mortem specimen showing upper esophageal sac, hypertrophied and dilated. Probe passes through trachea and tracheoesophageal fistuIa at bifurcation of bronchi into Iower end of esophagus which opens into stomach. Diffuse puI- monary hemorrhage.

pathognomonic picture easiIy understood from the underIying anatomica defects. The foIIowing is a short summary of the symptomatoIogy :

I. AbsoIute inabiIity to retain fluids 2. Regurgitation of fluid through the

nose and mouth associated with attacks of dyspnea, cough and cyanosis when put to breast or when gavaged. The attacks are constant and occur with each attempt to give fluids by mouth

3. Frothy ffuid at the nares and flow of saIiva from the mouth

4. FaiIure to pass a catheter into the esophagus more than IO to 12 cm. from the Iip margin

3. Air in the stomach if a trachea1 fistuIa is present

6. Moist ra1e.s throughout both Iung fieIds

7. GraduaIIy increasing temperature with deveIopment of bronchopneumonia

8. Increasing dehydration, Ioss of weight, and maInutrition

9. InevitabIe death within two weeks usuaIIy from bronchopneumonia

I o. Associated congenita1 anomaIies.

PATHOLOGY

Autopsy report, Case I, Dr. Thomas Robert- son (Fig. 5): There was a bIind pouch of the upper end of the esophagus 4.5 cm. in Iength which was hypertrophied and diIated. There was a tracheoesophageal fistuIa with the open- ing of the fIstuIa at the point of bifurcation of the trachea. There were no congenita1 anomaIies of the heart except the anatomicaIIy patent foramen ovaIe and ductus arteriosus which is expected at this time of Iife. The right heart was greatIy diIated, associated with the puI- monary congestion, in turn associated with the aspiration of &ids from the stomach. There was a supernumerary thumb which had been amputated. The inability to void might have been due to a marked phimosis with a Iong prepuce. The meatus was patent with no anomaIies. Each ureter was somewhat diIated and tortuous, and the kidney pelves were sIightIy diIated. The urinary bIadder was distended.

Autopsy report, Case II, Dr. Warren C. Hunter.

The esophagus ended in a bIind pouch 3 cm. from the point of origin, terminating in a fibrous cord. In the non-CartiIaginous portion of the trachea, beginning about 2 cm. above the carina, there is seen a crescent-shaped opening, which on probing is found to Iead to a tube which on dissection upward from the stomach proves to be the esophagus. There was nothing grossIy visibIe in the Iungs sug- gestive of a pneumonic process, but microscopic sections reveaIed an early bronchopneumonia. There were no associated abnormalities.

NEW Smms VOL. XXII, No. 2 Mathieu & Goldsmith&Atresia American Journal of Surgery 237

GROSS PATHOLOGY

There is a marked simiIarity in the gross findings of a11 cases of congenita1 atresia of the esophagus with tracheal fistula. The upper end of the esophagus ends in a bIind pouch and is usuaIIy 3 to 4.5 cm. Iong. The proxima1 portion of the esoph- agus is diIated and hypertrophied to a diameter of I cm. or more. The norma esophagus in infants measures about 4 to 6 mm. This hypertrophy may be expIained on the basis of futiIe attempts of the infant to swaIIow amniotic fluid or as evidence of abnorma1 growth.

The Iower portion of the esophagus is norma in its entrance into the stomach. The upper segment, however, narrows its Iumen to about 2-4 mm. and enters the trachea at or very near the bifurcation. Th e point of entrance into the trachea may take place 0.5-2.0 cm. above the bifurcation. A few cases have been re- ported where the fistuIa existed between the esophagus and a bronchus. There may, however, be an overIapping of the upper and Iower portions.

The two segments of the esophagus are usuaIIy separated by I cm. of fibrous tissue. Associated anomaIies, of which the most common is atresia ani, are frequentIy found. Congenital defects involving the kidneys, extremities and Iips have aIso been noted. The supernumerary thumb and phimosis noted in Case I were the onIg associated defects noted in the 2

cases here reported. Because of the desire to preserve the

gross structures of such an anomaIy, a study of microscopica pathoIogy has not been made in most cases. GeneraIIy speak-

ing, from those who have reported the histology on these cases, the extreme ends of the esophagus appear normaI in struc- ture. RosenthaI finds that the transition from trachea1 structures in the proximal end of the fistuIa to esophagea1 structures in the distal end of the esophagus is gradua1.

With the gradua1 deveIopment of de-

hydration and inanition and the over- flowing of regurgitated materia1 into the lungs, the Iungs become an idea1 Iocation for the deveIopment of bronchopneumonia. If the infant has weathered every attack of regurgitation with its accompanying dysp- nea and cyanosis, it must inevitabIy faI1 victim to pneumonia.

PROGNOSIS AND TREATMENT

Up to the present time every case of congenital atresia of the esophagus associ- ated with trachea1 fistuIa has been fata1. No method has yet been introduced which can successfuIIy carry on the nutritiona demands of the infant. Recta1 feeding and intravenous gIucose as an adjunct prove inadequate in promoting growth and nu- trition. Surgery must be heId forth as the onIy optimistic procedure for Iessening what is now an inevitabIe death.

Gastrostomy has been done in many cases with the idea of proIonging Iife to a point where Iater surgery may be effective. This has been proved inadequate, however, since flooding of the Iungs has occurred in those cases associated with fistuIa. Jeju- nostomy is unsatisfactory since it is prac- ticaIIy impossibIe to feed an infant by this method. Ritcher12 in 1913 advised gastros- tomy with cIosure of the upper end of the Iower portion of the esophagus. He reports 2 cases. One baby died of operative shock, the other Iived twenty-four hours. At aut.opsy no fluid was found in the Iungs of his second patient. Brenneman13 feeIs that intrathoracic surgery hoIds the great- est hope for reIief of congenital atresia of the esophagus, stiI1 mindfu1, however, of the insurmountabIe barrier to be overcome and the pediatric Iimitations of such a case so treated.

Brenneman’s phiIosophy to\vards such cases is broad and deep. It is worthy of repetition in toto:

I%.hen one considers on the other hand that nearlJ7 all of these infants have other anoma- lies, that practicaIly a11 of them either have bronchopneumonia or 41 aImost inevitabIy

238 American Journal of Surgery Mathieu & Goldsmith-Atresia NOVEMBER, 1933

get it if the upper portion of the esophagus is not drained into the Iower portion, that a “restitution ad integrum” of the esophagus does not yet seem feasibIe, that without this, Iife, even if possibIe, wouId be intoIerabIe, that the chiId couId probabIy not be made to Iive even if successfuIIy operated on, for pediatric reasons; that no such infant has ever lived, no matter how treated, and finaIIy, that parenta sentiment must be weighed heaviIy in the baIance, then if one, after carefu1 counse1 with the parents, decides to Iet the patient die as peacefuIIy and painIessIy as possible, one need Iose no sIeep because of that decision.

REFERENCES

I. DURSTON, G. Trans. No. 65 Pbilos. Ann 1670 Coil. Acad. Part Etrang. 1755 T P, p. 288.

2. ROSENTHAL, A. CongenitaI atresia of the esophagus with trachea-esophagea1 fistuIa. Arch. Patbol.,

I.

6.

7.

8.

9.

IO.

II.

12.

13.

14.

12: 756, 1931. 3. GIBSON, T. The Anatomy of Humane Bodies 15.

Epitomized. Ed. 6, 1703. 4. PL.ASS, E. D. Congenital atresia of the esophagus

with trachea-esophageal fistula: associated with

fused kidney. Jobns Hopkins Hosp. Rep., 18:

2.59, ‘9’9. GRIFFITH, J. P., and Lavenson, R. S. Congenital

maIformation of the esophagus with report of a case. Arch. Pediat., 26: 161, ;go9. _

CAUTLEY. E. Malformation of the esonhafms. &it 1 Y

J. Dis. Cbild., 14: I, 1917. BALLONTYNE, J. W. ManuaI of Antenatal PathoI-

ogy and Hygiene. Edinburgh, Green, 1904, p. 462.

KREUTER. Cited by SchriddIe, H.g SCHRIDDLE, H. Neber EpitheIproIiferationen in der

menschIichen Speiserohre. Vircbows Arch. f. patb. Anat., 191: 178, 1908.

KEITH, A., and SPICER, J. E. Three cases of maI- formation of the trachea-esophagea1 septum. J. Anat. ti Pbysiol., 41: 52, 1906.

ZEIT, F. R. CongenitaI atresia of the esophagus. J. Med. Research, 22: 45, 1912.

RICHTER, H. M. Congenital atresia of the esopha- gus; an operation designed for its cure. Surg. Gynec. Obst., p. 397, Oct. 1913.

BRENNEMAN. J. CongenitaI atresia of the esophaaus. Am. J. D&. Cbildyen, 16: 143, 1918. - -

ROSENTHAL, A. H., and HIMMELSTEIN, U. Review of former articIe of RosenthaI’s. Arch. Pediat.,

49; 444-462, 1932. GRAHAM, R. CongenitaI atresia of the esophagus.

Calijornia ti Western Med., 36: 180-183, 1932. (This reports two additiona cases with x-ray and pathoIogica1 reports.)

CONCLUSION OF DR. BALDWIN’S ARTICLE*

In cIosing wounds where avoidance of scars history of the Academy the “exhibit” was is especiaIIy desirabIe; it is soft, is very easiIy roundly appIauded. A IittIe Iater photographs twisted or tied, and Ieaves a practicaIIy invisi- were taken showing the mouth cIosed, and ble scar at the point of transfixion. aIso with the Iower jaw sIightIy dropped. In

These stitches were removed at the end of fuI1 yawning there is some deformity of the two fuI1 weeks, heaIing having taken pIace Ieft angIe, but under ordinary circumstances without a particIe of infection. The case was there is no noticeabIe defect of any kind. presented to the CoIumbus Academy of Medi- Correspondence with Dr. John Staige Davis, tine May I, a IittIe Iess than four weeks after Professor of PIastic Surgery at Johns Hopkins operation, as an iIIustration of Shakespeare’s University and author of a weII-known text- weII-known dictum that “There’s a divinity book on pIastic surgery, indicates that this is which shapes our ends, Rough hew them how the first instance in which a vermiIion border we wiI1.” For the first time, probabIy, in the has been secured on such a transplant.

*Continued from p. 232.