CONGENITAL ATRESIA OF THE ESOPHAGUS WITH TRACHEO-ESOPHAGEALFISTIJLA, DUODENAL ATRESIA, AND OTHER ANOMALIES

A CASE REPORT*

WILLIAM C. MCGARITY, M.D., OSLER A. ABBOTT, M.D.,AND LON W. GROVE, M.D.EMORY UNIVERSITY, GEORGIA

FROM THE \W'HITEHEAD DEPARTMENT OF SURGERY, EMORY UNIVERSITY SCHOOL OF MEDICINE, EMORY UNIVERSITY

PLASS3 IN 1919, presenting a survey ofthe literature on congenital anomalies of theesophagus, was able to collect only 136completely verified cases recorded of con-genital atresia of the esophagus. Accordingto reports in the more recent literature,however, this anomaly occurs once in ap-proximatelv 2500 births, or with about thesame frequency as harelip and cleft palate.'These reports are substantiated in part bythe fact that, between 1939 and 1947, 113patients with this condition were admittedto the Children's Hospital in Boston.4

In the most common form of the anomaly,a tracheo-esophageal fistula is associatedwith the atresia. A fistula may connect thetrachea with either the proximal or distalesophageal segments, and rarely with bothsegments. In approximately 94 per cent ofcases the fistula communicates with thelower esophageal segment.4 All reportscomment on the frequency of the occur-rence of other anomalies in association withesophageal atresia. In Plass' series the pres-ence or absence of other anomalies wasnoted in 94 cases, and in 59 of these, or 62.8per cent, disorders vere present in otherorgans. Atresia of the anus was the asso-ciated anomaly found most frequently.Ladd and Swenson,2 reporting on a seriesof 114 cases, have shown in tabular form(Table I) the incidence of various types of

* Submitted for publication August, 1951.

associated malformations, some of whichwere multiple in a single patient. It is ad-vised that early recognition of such asso-ciated anomalies is important, and shouldbe considered in the immediate treatmentof the patient.

TABLE I.-Anomalies Associated with Atresia ofthe Esophagus.

Type Incidence

Atresia or stenosis of the small intestine 7Meckel's diverticulum ......... ........... 8Malrotation . . 4Imperforate anus ........................ I1Fistula of the rectum .................... 4Anomalies of the heart and aorta.......... 20Congenital anomalies of the urinary system 13Miscellaneous ........................... 24

The following report presents a case inwhich congenital atresia of the esophaguswas found in association with tracheo-esophageal fistula, duodenal atresia andother anomalies.

CASE REPORT

Master M. F., age 24 hours, was admittedto Emory University Hospital at 1:00 A.M., March14, 1950. He was a full term infant who had anormal spontaneous delivery and had cried wellat birth. Soon after birth, however, he was notedto have irregular and difficult respiration. Onphysical examination auscultation of the epigastricarea revealed a loud rush of breath sounds comingin and out of the stomach corresponding to inspira-tion and expiration. There was also palpablechange in the size of the stomach with respiration,although there was no visible distention of theabdomen.

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A No. 12 F. soft rubber catheter inserted intothe esophagus under fluoroscopic guidance met an

obstruction just above the level of the bifurcationof the trachea. A small amount of Lipiodol was

injected, and a roentgenogram taken at this timedemonstrated a blind upper esophageal segment(Fig. 1). Roentgenograms of the abdomen showedthe stomach and first portion of the duodenum tobe distended with air (Fig. 2). The intestine,

used with transection of the second, third andfourth ribs at their angle. By developing an extra-pleural plane of dissection, the mediastinal struc-tures were revealed. The upper esophagus was dis-sected from the surrounding structures and theblind esophageal pouch located about 2.5 cm.

above the level of the carina of the trachea. Withfurther dissection the distal esophagus was foundto be attached to the left main stem bronchus on

I11. 1VI(. 2

FIG. 1.-An oblique view of the upper thorax demonstrating the blind esophageal pouch.The trachea air column is outlined.

FIG. 2.-A right posterior oblique view of the abdomen, demonstrating marked distentionof the stomach and first portion of the duodenum. The duodenum is outlined for better visual-ization. No intestinal air is seen beyond the duodenum.

however, showed no air pattern beyond the firstportion of the duodenum. The diagnostic impres-sion was the presence of congenital atresia of theesophagus associated with a tracheo-esophageal fis-tula which communicated between the trachea or

bronchus and the lower esophageal segment, andobstruction of the first portion of the duodenum.

Ten hours after admission to the hospital, an

exploratory thoracotomy was carried out undercyclopropane anesthesia, using a tight fitting facemask. With the patient in the prone position theright posterolateral extrapleural approach was

its postero-inferior wall about 1.5 cm. distal to thebifurcation of the trachea. Agenesis thus produceda gap of approximately 4 cm. in the esophagus.

The distal esophagus was doubly ligated at itsjunction with the left main stem bronchus with 000silk, and then was cut just below the ligatures. Itwas necessary to mobilize completely the esophagusfrom the level of the diaphragm up to the fistulouscommunication and also to mobilize the upperesophageal segment as high in the neck as possible.A primary anastomosis between the proximal anddistal ends of the esophagus was performed, fol-

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lowing which there was a great deal of tension onthe suture line of the anastomosis. The upperesophagus was sutured carefully in several placesto the peritracheal tissues. A No. 10 F. soft rubbercatheter which had been inserted throughout thelength of the esophagus during the anastomosiswas allowed to remain for gastric drainage pendinggastrostomy. A thoracotomy tube, using a No. 14mushroom catheter, was inserted through the rightposterolateral chest wall for closed drainage. Theincision was closed in layers with fine silk. Thepatient withstood the procedure well and had asmooth postoperative course the night followingthe operation.

rT AND GROVE Annal6 of SurgeryA p rNi I1, 19952

brought out through a stab wound in the upperright quadrant. The upper midline incision wasclosed in layers.

The patient's postoperative course was remark-ably smooth for 4 days following the gastrojejunos-tomy. The thoracotomy tube was removed 3 daysfollowing the first operation. The gastrostomy tubedrained well. Bile was noted to be draining fromthe tube.

On March 20 the patient was started on gas-trostomy feedings. After approximately 3 feedingshe had a massive aspiration followed b) fulminat-ing bilateral pneumonia. Roentgenographic studiescarried out on this date revealed consolidation of

FIG. 3.-Postmortem specimen, revealing dilatation of the stomach andfirst portion of the duodenum, atresia of the second portion of the duodenumjust proximal to the entrance of the common duct, a Meckel's diverticulumof the distal ileum, and the sites of gastrostomy and gastrojejunostomy.

The following day an exploratory laparotomywas carried out under cyclopropane anesthesia. Anupper midline incision was used. On entering theabdominal cavity, the stomach and first portion ofthe duodenum were markedly dilated. Since bilewas aspirated from the third portion of the duo-denum, it was evident that the obstruction wasabove the ampulla of Vater; however, the definitesite of obstruction was not visualized or palpated.An antecolic gastrojejunostomy was performed.After completion of the gastrojejunostomy, a gas-trostomy was carried out by inserting a small mush-room catheter through a 1 cm. longitudinal incisionin the mid-anterior wall of the stomach. The cathe-ter was held in place bv a purse-string suture and

the right lung throughout most of its volume. Thereappeared to be some fluid in the right pleural space.A needle was inserted into the right posterior lowerchest and a yellowish purulent fluid was obtained.It was thought at this time that the patient possiblyalso had a leak at the site of esophago-esophagealanastomosis. Closed thoracotomy tube drainage wasagain instituted. The patient's course, however,was steadily downhill and he expired on March 21.

A postmortem examination revealed the patientto have multiple anomalies (Fig. 3). Within thechest a necrosis of the esophago-esophageal anasto-mosis was found to have occurred across the an-terior surface of the anastomosis with a resultantleakage. There was evidence of mediastinitis,

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empyema of the right chest, and extensive bilateralpneumonia. The area of fistula repair in the leftmain stem bronchus, and the gastrojejunostomyand gastrostomy, were in good condition. The firstportion of the duodenum was markedly dilated,being about three to four times normal size. Anatresia of the second portion of the duodenum wasfound just above the entrance of the common ductinto the duodenum. The atresia measured 1 cm. inlength. Microscopic serial sections of the duode-num involved by the atresia showed fibrous tissuesurrounded by pancreatic tissue. There was nolumen or evidence of mucosa in the area of theatresia. The biliary tree and ampulla of Vaterwere patent. A Meckel's diverticulum measuringone centimeter in length was also found 10 cm.above the cecum on the antimesenteric border ofthe ileum. Congenital heart disease manifested bythe Eisenmenger complex, an interatrial septaldefect, a bicuspid pulmonary valve, and a patentductus arteriosus, was also discovered.

DISCUSSION

We now believe that in a patient with acongenital esophageal atresia and tracheo-esophageal fistula associated with a duo-denal atresia, gastrojejunostomy and gas-trostomy should probably be performedprior to correction of the esophageal con-dition. The gastrostomy tube would be leftopen for the purpose of deflating thestomach.The second procedure, closure of the

tracheo-esophageal fistula and correction ofthe esophageal atresia, should be carriedout in approximately 24 to 48 hours follow-ing the first operation. Recent advances indevising reconstructive procedures consid-erably decrease the demand for a primaryend-to-end esophageal anastomosis. Cer-tainly the situation would need to be idealin regard to satisfactory circulation andcomplete absence of tension on the sutureline if one is to recommend primary eso-phageal anastomosis in a patient with duo-denal atresia. Not only is there danger tothe esophageal suture -line from the highintragastric pressures consequent to theearly postoperative period of gastro-enter-ostomy, but the relaxed state of the crico-pharyngeal muscle in these cases predis-

poses to massive aspiration. Massive aspir-ation followed by pneumonia was respon-sible for this child's death.The authors recommend that when

tracheo-esophageal fistula with esophagealand duodenal atresia coexist that no attemptat primary esophageal anastomosis be made,believing it better to transect the fistula,adequately closing the fistulous openings,and to carry out an exteriorization of theupper segment of the esophagus in theneck. A subsequent high esophagogastros-tomy should be performed at a later date,preferably after the age of two years.

SUMMARY

1. The case of an infant having eso-phageal atresia coexistent with tracheo-eso-phageal fistula and duodenal atresia, as wellas other anomalies, has been presented.

2. A loud to-and-fro rush of air withinthe stomach with each respiration appearsto be an important physical sign in thiscombination of anomalies.

3. The roentgenographic aspects of thisdisease entity are described.

4. It is recommended that this complexsituation be treated first by a laparotomywith gastrostomy and gastro-enterostomy,and secondly by a thoracotomy wit-h clo-sure of the tracheo-esophageal fistula andexteriorization -of the upper esophagealstump.

5. Procedures to reconstruct the conti-nuity of the gastro-intestinal tract would bedone electively after the age of two years.

BIBLIOGRAPHYBigger, I. A.: The Treatment of Congenital

Atresia of the Esophagus with Tracheo-Eso-phageal Fistula. Ann. Surg., 129: 572, 1949.

2 Ladd, W. E., and Orvar Swenson: EsophagealAtresia and Tracheo-Esophageal Fistula. Ann.Surg., 125: 23, 1947.

3 Plass, E. O.: Congenital Atresia of the Esoph-agus with Tracheo-Esophageal Fistula Asso-ciated with Fused Kidney. Johns HopkinsHosp. Reports, 18: 259, 1919.

4 Swenson, Orvar: The Diagnosis and Treatmentof Atresia of the Esophagus and Tracheo-Eso-phageal Fistula. Pediatrics, 1: 195, 1948.

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