Paper - Congenital atresia of the oesophagus
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Franklin RH. Congenital atresia of the oesophagus. (1948) Ann R Coll Surg Engl. 2(2): 69–79.
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Congenital Atresia of the Oesophagus
Hunterian Lecture delivered at the Royal College of Surgeons of England
on 16th January, 1947
R. H. Franklin, M.B., B.S., F.R.C.S.,
Department of Surgery, Postgraduate Medical School of London
Introduction - Pathological Types
So many varieties of congenital malformation of the oesophagus have been described that the true nature of the least rare variety of all is often overlooked; this is an atresia or interruption in the continuity of the oesophagus, with, usually, a ﬁstula between the trachea and the distal segment. In 1929 Vogt made a classiﬁcation of the different types of atresia :—
- TYPE I.—Complete absence of oesophagus.
- TYPE II.—Atresia of the oesophagus with an upper and lower oesophageal segment, each ending in a blind pouch.
- TYPE III.—Atresia of the oesophagus with tracheo-(esophageal ﬁstula.
(A) With ﬁstula between upper segment and trachea. (B) With ﬁstula between lower segment and trachea. (C) With ﬁstula between both segments and trachea.
Eighty per cent. of all cases of atresia are of Type III(B), Fig. 1. That is to say, the upper segment ends blindly at about the level of the vena azygos arch, or 10 to 12 cm. from the anterior alveolar margin; it is dilated and hypertrophied and remarkably constant in size and shape. The lower segment shows more variation; it springs from the back of the trachea, usually within 1 cm. of the bifurcation, or from the bifurcation itself; within these limits it varies considerably, so that it may overlap the upper segment or it may be separated from it.
The lower segment is nearly always thin-walled and usually about half the calibre of the upper segment. It may be of nearly uniform diameter throughout its length or it may taper as it approaches the trachea.
These characteristics and variations are of great importance in determining the feasability of anastomosis.
Fig. 1. (a) Type III(B), the most common type, accounting for 80 per cent. of all cases. (b) Next most common type. (c), (d) and (e) All rare.
Type II accounts for most of the cases which do not fall into the characteristic group. Type HI(A) and Type III(c) are rare.
Cameron Haight encountered an example of a single ﬁstula between oesophagus and trachea which was not associated with atresia.
Gibson, Physician-General to the Army and a grandson of Oliver Cromwell, described this condition in 1697 and it was mentioned by Durston some 27 years earlier.
About 250 years elapsed before successful methods of management were devised and carried out. The subject was not forgotten during this time. Martin published a case report in 1821, and in 1884 MacKenzie was able to collect records of 43 cases. In 1910 Sir Arthur Keith searched the museums of London and found 14 examples of atresia. He described accurately what we now know to be the most usual type, and gave a satisfactory account of the developmental errors underlying the condition.
The only type of operation carried out at this period was a simple gastrostomy.
Honourable exception must be accorded to Richter, who attempted a rational operation on two infants in 1913, a year memorable for Torek’s successful removal of the thoracic oesophagus for carcinoma.
By 1933 some 300 cases had been reported in the literature; most of the reports emphasised the rarity and hopelessness of the condition, while a few of the more adventurous expressed the pious hope that the condition might one day yield to surgery.
Two popular misconceptions have discouraged attempts at treatment, ﬁrst the alleged extreme rarity of the lesion, and second the mistaken idea that this anomaly is usually associated with other and equally serious malformations.
The second misconception is the more insidious in that it gives that ﬁnal stamp of hopelessness to what is already a very diﬂicult surgical problem. It is true that Plass (1919) foundthat about half the recorded cases show associated anomalies. But it must also be remembered that in 45 consecutive cases, Cameron Haight found that only one patient had an additional deformity which was incompatible with life.
The possible presence of other abnormalities, particularly imperforate anus, must be recognised, but this recognition must not be used as an argument for regarding the condition as hopeless.
Most authors have commented on the extreme rarity of the condition —the popular ﬁgure given being 1 in 50,000 births. Guthrie (1945), searching the material at the Royal Hospital for Sick Children, Glasgow, found that between 1915 and 1944, in 6,916 consecutive post mortem examinations, malformations of the oesophagus were found in 38 subjects, and no fewer than 24 of these provided examples of atresia (1 in 284 autopsies). Six other patients who did not come to autopsy presented suggestive clinical features. At the Postgraduate Medical School of London, from 1935 to 1942, four cases of atresia were found in 10,543 deliveries, or one in 2,635 deliveries. When it is considered that nearly every one of the autopsy cases investigated by Guthrie showed pulmonary complications of great severity, it is reasonable to assume that a number of these infants succumb without the true nature of the lesion being recognised.
The Surgical Problem
Attempts at feeding ﬁll the blind upper segment so that its contents spill over into the trachea to evoke attacks of cyanosis, and, before long, aspiration pneumonia. Even if feeding is avoided, the blind upper sac ﬁlls with mucus and saliva unless aspiration is carried out at frequent intervals. Gastrostomy feeds too ﬁnd their way into the lungs through the distal oesophagus and its tracheal ﬁstula, unless the anomaly is one of the rare forms in which there is a blind lower segment. Apart from the fact that gastric contents may pass into the lungs with fatal results, air passing from the trachea into the stomach sometimes causes considerable gastric distension. A type of spurious or see-saw respiration may even be observed, air passing backwards and forwards from the lungs to the stomach by way of trachea, ﬁstula and oesophagus ; this gastric respiration produces a rapidly increasing anoxia.
In an attempt to save these infants, Steele carried out gastrostomy in 1888. Others followed his example, but all gastrostomies were unsuccessful because of the regurgitation into the lungs. Brenneman (1918) hoped to overcome this difﬁculty by substituting a jejunostomy for gastrostomy, but again without success. Richter, in 1913, recognised the importance of the ﬁstula and advocated transpleural ligature of the lower oesophagus together with gastrostomy. His two cases were unsuccessful, and it must be noted that he made no provision for keeping the blind upper segment empty. Direct anastomosis by means of the Murphy button was advocated by Von Hacker in 1926 (quoted by Lanman, 1940), but without success.
Fig. 2. Stages in the development of the operation.
(a) Simple gastrostomy.
(b) Gastrostomy + ligature at cardiac end of the oesophagus.
(c) Formation of a gastric spur.
(d) Formation of stomach tube together with ligature of lower oesophagus.
(e) Gastrostomy + direct approach to the ﬁstula and ligature.
(f) Exteriorization of both segments. (g) Gastrostomy + oesophagostomy and ligature of ﬁstula.
(h) Present operation—direct approach and anastomosis.
By 1929 there was more general realisation of the nature of the problem, and its solution was seen to require 1- 1. Prevention of the blind upper segment from ﬁlling up and overﬂowing.
II. Prevention of the regurgitation of gastric contents through the ﬁstula into the trachea.
III. Successful feeding of the infants.
During the next few years all sorts of methods were adopted to achieve these results, and the names of Mixter, Leven, Gage and Ochsner, Gamble and Ladd are all associated with multiple operations which include cervical oesophagostomy, various types of gastrostomy, transection of the stomach and ligature of the ﬁstula. Out of all these attempts only two were completely successful at this time; the staged operations performed by Ladd in 1939, and Leven in 1940.
The multiple-stage operation presents the patient and the surgeon with so difficult a succession of hurdles even if the infant survives the early procedures, that Lanman ventured a primary anastomosis by the extrapleural route in 1936, but none of his patients survived.
Haight and Townsley reported in 1941 the ﬁrst completely successful direct anastomosis, and the tendency now is to favour direct anastomosis wherever possible. The development of the operation is shown in Fig. 2.
The transpleural and extrapleural routes have each had their supporters. Haight and Townsley used the latter in their ﬁrst successful case. The anastomosis is less diﬂicult by the transpleural route and the operation seems to be well tolerated, but up to the present time this approach has, generally speaking, proved unsuccessful, although Singleton and Knight report the case of a patient who lived for over seven months following the transpleural operation, and whose death then was probably due to a ﬁstula of the trachea, unsuspected during life, with the upper blind segment of the oesophagus (Vogt’s Type III(c)).
Impressed with the diﬂiculties which I experienced in carrying out the extrapleural operation, both in the postmortem room and in the operating theatre, I concentrated at ﬁrst on the right transpleural approach, but have been forced to the conclusion that in the anastomosis of such a delicate structure as the infant’s oesophagus, it is diﬂicult to avoid leakage, and if the leak occurs inside the pleural cavity it is likely to be fatal. My own experience, which has impressed me with the value of the extrapleural procedure, has been gained in the following cases :—
OPERATIONS ON CASES or CONGENITAL ATRESIA or OESOPHAGUS 1938-1946. FRANKLIN
Sex and Birth Case Date Age Weight Procedure Survival Remarks -(Hours) and Type
4:417 1 1:
1 21.11.38 M. 61b. 7oz. Ligature of cardia and 7 days Silk ligature slowly cut 72 III(B) gastrostomy through and re-established . the fistula 2 16.10.41 M. Prem. 41b. 5oz. Transpleural (R). 17 hours Pneumonia 84 III(B) Anastomosis 3 31.5.42 F. Prem. 51b. 8oz. Transpleural (R). Fistula Died on Massive collapse of both 148 III(B) ligated_ and divided, table lungs tube tied into lower segment 4 2.11.43 F. Prem. 41b. 3oz. Transpleural (R). 5} days Leakage of anastomosis. 72 III(B) Anastomosis Case reported (O’REILLY, l N.. FRANKLIN. R. H.. and DALEY, M. D.. 1944) 5 6.11.45 F. Prem. Transpleural (R). 43 hours _ Pneumonia III(,a) Tube tied into each segment ' 6 13.1.46 F. | 51b. 8oz. Transpleural (R). 52 hours Pneumonia 28 III(B) Fistula ligated and | divided. Gastrostomy 7 21.2.46 F. 51b. 6oz. Extrapleural approach 27 hours Pulmonary collapse I 120 III(n) attempted. Pleura tore badly._ Anastomosis with difﬁculty 8 i27.l0.46 F. 71b. 2oz. Transpleural (R). 25 hours Consolidation both lungs. I 72 II No ﬁstula. Lower scg- Patent ductus arteriosus. ment ended 1-5 cm. No gastric gas bubble was above diaphragm. seen at X-ray—this should I Gastrostomy | have led to the correct diagnosis of type 9 10.1.47 F. 71b. 7oz. Extrapleural (R). Alive and Gastrostomy carried out 72 HI(B) Anastomosis well 6 days after anastomosis. Gastrostomy tube re. moved after 12 days. Infant now takes food normally and has made I good progress. (Previously re orted, FRANKLIN, R. .. 1947) 10 24.4.47 F. 61b. 7oz. Extrapleural (R). Alive and Gastrostomy _was not 48 III(a) Anastomosis well necessary in this case. The
infant made a smooth H recovery and has since progressed well. (Previously ilwegizggted. FRANKLIN, R. H..
Cyanosis associated with excess of mucus in the nasopharynx may be noted at birth in many normal infants. Improvement in these occurs after aspiration, but the child suffering from atresia has recurrent attacks of cyanosis which are usually severe enough to excite notice, but which are often attributed to other causes, such as tentorial tears and atelectasis. When feeds are started all the symptoms are accentuated, the child is often avid for its feed and sucks strongly, only to be overwhelmed by an attack of spluttering and cyanosis, accompanied by the complete regurgitation of the feed. Aspiration of the nasopharynx effects an immediate improvement, but the symptoms recur as soon as feeding is attempted again.
These symptoms are so striking and alarming that they seldom escape notice, and it cannot be too strongly emphasised that such a train of symptoms usually indicate oesophageal atresia. Midwives and others who have the care of infants in the ﬁrst hours of life, should be taught that atresia is the commonest cause of persistent choking and cyanosis in the newborn child.
Once the suspicion of atresia has been raised, all mouth feeding is stopped and the nasopharynx is aspirated at frequent intervals.
To conﬁrm the diagnosis, a well-lubricated rubber catheter is passed through the mouth and down the oesophagus. Its arrest 10-12 cm. from the anterior alveolar margin is practically diagnostic of atresia.
Final conﬁrmation of the diagnosis is radiological, but the practice of giving a Barium swallow is to be condemned. The emulsion is almost certain to spill over into the lungs and the child’s slender chance of survival is still further reduced. The best method to adopt is as follows :—
The infant is screened and a careful assessment is made of the condition of the lungs; the stomach and intestines are then examined for the presence of air. If air is present in the stomach and intestines below an (esophageal atresia it is proof that a ﬁstula exists between the lower segment and trachea. The converse is usually, but not invariably, true.
A catheter is now passed into the upper segment and l to 2 cc. of lipiodol is gently introduced under direct observation against the screen.
The appearance of the blind segment is characteristic, and as soon as it has been observed and the rare presence of a ﬁstula in the upper segment excluded, the lipiodol is withdrawn (Figs. 3, 4 and 5). Bronchoscopic examination has been used to inspect the ﬁstula and so conﬁrm the diagnosis (Pilcher). Apart from the passage of a catheter into the blind sac and frequent aspiration, the important features in the management of the diagnostic stage are negative rather than positive. The chief safety factors are the avoidance of any further feeding and of unskilled attempts at X-ray diagnosis.
Management after the Diagnosis has been Conﬁrmed
The complete diagnosis not only conﬁrms the presence of atresia, but in many cases discloses the precise nature of the deformity. It must be emphasised once more that 80 per cent. of the cases fall into the characteristic group of (esophageal atresia with ﬁstula between trachea and lower oesophageal segment.
The preparation of the patient for operation requires the full collaboration of an expert paediatrician, as well as the whole-time services of an intelligent and conscientious nurse. In fact, without these two essentials before and after operation, any surgical intervention is almost certain to fail.
The three essentials in the pre-operative period are : to keep the blind sac empty by aspiration, to improve the state of the lungs by changing the child’s position frequently and systematically, and to administer the requisite ﬂuid parenterally. The ﬂuid requirement is difficult to assess, and giving too much is probably more dangerous than giving too little.
Fig. 3. X-ray showing large air bubble in stomach and blind upper oesophageal segment filled with iodised oil.
Blind upper segment ﬁlled with iodised oil. Care has been taken not to allow any to spill over into the larynx.
Fig. 5 An earlier case in which the importance of great care in X-ray diagnosis was not fully appreciated and the iodised oil has spilled over into the lungs. Although the oesophagus was restored and is seen to be functioning, the_pulrnonary condition contributed to the unsuccessful result in this case.
The child is secured in a prone position over a rubber hot-water bottle, half-ﬁlled with warm water. A small pad is placed under the right shoulder
‘and the head is turned to the right. Local anaesthesia (1 per cent. Procain)
is used for the skin incision and the anaesthetist concentrates on keeping the upper oesophageal segment empty and administering oxygen unde pressure from time to time. T
The incision is curved (Fig. 6), starting 1 cm. lateral to the spine, over the second rib, passing downwards parallel to the spine over the third and fourth ribs and then outwards crossing the ﬁfth rib obliquely and ending over the sixth rib. Part of the ﬁfth rib is resected subperiosteally, extreme care being necessary to avoid opening the pleura. The correct extrapleural plane having been found, the incision is deepened through the chest wall with division of the fourth, third and second ribs and their intercostal bundles. The pleura is carefully displaced until the vena azygos arch is seen. This usually marks the lower end of the upper blind segment and it is divided between ligatures with great care.
Scar after mediastinal incision. Fig. 6
The lower oesophageal segment emerges from the posterior surface of the trachea or from the bifurcation itself. Gentle movement of the catheter in the upper segment helps in identiﬁcation. The ﬁstula is ligatured close to the trachea and divided, with conservation of as much as possible of the lower segment, and traction sutures are introduced into each segment to avoid handling them unnecessarily.
Anastomosis is undertaken if it seems feasible without undue tension, and it is performed with the use of ﬁne silk and small, curved, eyeless round-bodied needles. Various methods of anastomosis have been described, but none of them is easy. The structures are delicate and readily damaged, and there is a great tendency for the stitches to cut out. A catheter introduced into the oesophagus from the mouth during the later stages is of great help. On completion of the anastomosis the area is well dusted with penicillin powder, the wound is closed around a small rubber drain, and the oesophageal tube is removed.
If anastomosis has proved impossible, Humphreys has advised that the ﬁrst stage of a multiple-stage operation be carried out. A ﬁnger is passed along the back of the trachea into the base of the neck near the suprasternal notch. An incision is made between the right sternomastoid and the trachea, and through this the upper segment is drawn to establish a cervical oesophagostomy. If a ﬁstula is present it is ligated and divided at this ﬁrst stage, but failure to effect an anastomosis may be due to the absence of a ﬁstula ; the blind ends of the oesophagus being further apart when atresia is uncomplicated (Type II) than when a tracheo-oesophageal ﬁstula is present as well (Type III(B)).
The child is nursed in an oxygen tent and the same care is taken to aspirate the nasopharynx as before operation. A small blood transfusion (100 cc.) may be given, followed by an intravenous drip of N/5 glucose saline. The ﬂuid and salt balance must be controlled with great care and is best entrusted to a skilled paediatrician.
Immediately after the operation, feeds of penicillin solution (10,000 units) hourly are started by mouth. The mediastinal drainage tube is removed on the third or fourth day and the patency of the oesophagus is tested radiographically with iodised oil; if the reconstructed oesophagus conducts the oil without leakage, feeds of expressed breast milk are begun.
Gastrostomy is performed only if the swallow of iodised oil demonstrates a mediastinal leak; even if it is shown to be necessary by this criterion, its performance is best deferred until the seventh or eighth day after oesophageal repair.
Results in Other Centres Ha1'ght’.<' results to 1946 :—
45 consecutive patients.
42 had associated tracheo-oesophageal ﬁstula ; one additional patient had a tracheo-oesophageal ﬁstula without (esophageal atresia.
36 patients were explored.
26 patients were submitted to intrathoracic restoration of oesophagus.
9 patients survived operation from 6 months to 4% years.
Only one patient in this series had an additional anomaly which was incompatible with life.
Ladd’s results to 1944 :—
34 patients explored. 28 patients submitted to a staged operation ; nine living. 6 patients treated by primary anastomosis ; two living. Ladd’s ﬁrst success was a staged operation in November, 1939; an antethoracic oesophagus was constructed and the child was reported alive at the age of 4.5 years.
This paper is presented in the hope that midwives and paediatricians will regard all newborn infants who suﬂ"er from attacks of cyanosis and choking, together with the regurgitation of feeds, to be subjects of atresia of the oesophagus until the oesophagus can be shown, by the clinical and radiological methods described, to be normal.
If this attitude is inculcated and lethal attempts at feeding are stopped until the condition of the oesophagus has been determined by the simple passage of a catheter, we shall have more frequent opportunities of treating these little patients before aspiration pneumonia has jeopardised their chances of recovery.
The radiologist can contribute to the success of the operation by refraining from any form of examination which risks ﬁlling the lungs with opaque medium and the surgeon can prepare himself for a difficult operative procedure by a study of the varied pathology of (Esophageal atresia and of the methods which have proved successful in its treatment.
VOGT, E. C. (1929) Amer. J. Roentg. 22, 463.
HAIGHT, C. (1944) Trans. Amer. Surg. Ass. 62, 623.
———:(1944) Ann. Surg. 120, 623.
HAIGHT, C. and TowNsLEY, H. A. (1943) Surg. Gynec. Obstet. 76, 672.
HOLT, F. J., HAIGHT, C. and HODGES, F. J. (1946) Radiology 47, 457.
GIBSON, T. (1697) The Anatomy of humane bodies epitomized, 5th Ed. London, p. 239. MACKENZIE, M. (1884) Diseases of the throat and nose.
KEITH, A. (1910) Brit. Med. J. 1, 301.
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PLASS, E. D. (1919) Johns Hopkins Hosp. Report, 18, 258.
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——:(1941) Ibid. 10, 648.
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GAMBLE, H. A. (1938) Ann. Surg. 107, 701.
LADD, W. E. (1944) New Eng. J. Med. 230, 625.
SINGLETON, A. C. and KNIGHT, M. D. (1944) Ann. Surg. 119, 556.
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Carroll & Co.
O’REILLY, N., FRANKLIN, R. H. and DALEY, M. D. (1944) Arch. Dis. Child. 19, 131. FRANKLIN, R. H. (1947) Lancet 2, 243.
HUMPHREYS, G. H. (1944) Surgery 15, 801.
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