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Congenital anomalies of the esophagus
CONGENITAL
Congenital
DISEASES
Anomalies
ROBERT R. SHAW,
M.D.
AND DONALD L. PAULSON, M.D., Dallas, Texas
From tbe Department of Surgery of tbe University of Texas Soutbwestern Medical School and Baylor Hospital, Dallas, Texas.
ONGENITAL anomahes of the esophagus resuit from a fauIt in deveIopment at about the fourth week in the Iife of the embryo. A groove in the ventra1 waI1 of the primitive pharynx deepens and converts into the Iaryngotrachea1 tube. This tube is in intimate reIation to the upper part of the foregut, the Iengthening of which forms the esophagus. Incomplete separation of the trachea and esophagus, and faiIure of recanaIization of the esophagus resuIt in: (I) atresia of the esophagus with tracheoesophagea1 fistuIa, (2) atresia without tracheoesophagea1 f%tuIa, and (3) tracheoesophagea1 IistuIa without atresia. Other anomaIies of the esophagus are: (I) redupIications of the esophagus, (2) paraesophagea1 cysts, and (3) diverticuIa. Atresia of the esophagus with or without tracheoesophagea1 fistuIa is the most common anomaIy of the esophagus. Humphreys et aI. [I] report that it occurs in a ratio of approximateIy I :3,000 births. Its surgica1 correction is a diI%cuIt task, which taxes the ski11 and experience of the surgeon, anesthetist and hospita1 staff invoIved in the postoperative care. The reader is referred to exceIIent contributions that have been made by Lanman [2], Ladd and Gross [?I, Leven [4], Haight [5], Swenson [6], Gross [7], Humphreys et a1. [I], Potts [8] and others. UnIess corrected surgicahy, the anomaIy is incompatible with life since the infant cannot be fed. The earIy threat, however, is chieAy to the respiratory tract from aspiration of secretions from the mouth and reffux of the gastrointestina1 secretions through the Iower esopha-
C
American
Journal of Surgery. Volume 93. February.
of the Esophagus
1977
gus into the trachea. This latter danger is the greater one. During the period before the anomaIy is corrected the infant shouId be pIaced in a semi-upright position to aIIow gravity to overcome the tendency toward retIux. Nursing attendants shouId be vigilant in the aspiration of pharyngea1 secretions. There shouId be no immediate concern about the giving of parentera fIuids, since the newborn infant at term is pIethoric and we11 abIe to endure three or four days without fluids. DIAGNOSIS Atresia of the esophagus shouId be suspected when a newborn infant drooIs excessiveIy, chokes upon feedings and has cyanotic episodes. The diagnosis can be confirmed by the inabiIity to pass a catheter into the stomach and the VisuaIization of a bIind upper pouch on roentgenogram after putting opaque media (never barium) into the upper esophagus. The presence of air in the stomach and intestines as shown by the roentgenogram establishes the fact that a tracheoesophagea1 fIstuIa is present. The absence of air suggests that there is no fistula between the Iower esophagus and trachea but does not ruIe out this possibihty. Tracheoesophagea1 IistuIa without atresia shouId be suspected when a newborn infant has severe coughing episodes when fed. The fistuIa may be demonstrated by roentgenograms taken after opaque media has been pIaced in the esophagus with the infant in the prone position. ANATOMY OF
THE
ANOMALIES
Atresia of the esophagus with tracheoesophagea1 fistuIa, in which the Iower esophagus enters the posterior waI1 of the trachea, makes 196
CongenitaI
AnomaIies
FIG. IA. This roentgrnogram shows the typical anatomy of congenital atresia of the esophagus with tracheoesophagea1 fistula. Note smaIl tapered Iower segment communicating with trachea. (It is not advisabIe to use this amount of opaque media during the roentgenographic examination.)
of Esophagus
Flc;. IB. This is the same type of anomaly as shown in Figure IA. Note Iong upper blind pouch overIapping large Iower scgmcnt which forms a fistuta with the trachea.
Atresia of the esophagus without tracheoesophagea1 fistuIa is variabIe in the Iength of the upper esophagea1 pouch. (Fig. 2.) The Iower esophagus may vary from an atretic cord of tissue to a sharpIy tapering esophagus ending bIindIy in the posterior mediastinum adherent to but not communicating with the trachea. A variant of this anomaly is the congenita1 esophagea1 web Iocated at the mid-esophagus. TracheoesophageaI fistuIa without atresia is represented by a we11 developed esophagus which is intimateIy adherent to the trachealis muscle aIong the lower portion of the trachea with a fistuIous opening above the bifurcation. In rare instances two fistuIas may be present, with the upper segment as we11 as the Iower segment communicating with the trachea.
up 90 per cent of the anomaIies of this type. There is considerabIe anatomic variation in this anomaly. The upper esophagus, which is a blind pouch, is uniformIy thickened but may vary in Iength from a smaI1 pouch high in the thorax to a bIind segment extending aImost to the diaphragm. (Fig. I.) In Iike manner, the lower esophagus may be we11 developed and enter the posterior trachea1 waI1 with a patuIous opening as high as 2 cm. above the bifurcation of the trachea, or it may be a smaI1 underdeveIoped muscuIar tube which tapers sharpIy at its upper end to form a small fistuIa at the bifurcation of the trachea or at the upper portion of the Ieft main bronchus. Thus the two esophagea1 segments may overIap 2 to 3 cm. or there may be a gap of similar Iength between their two ends. When an overIapping esophagus is exposed at the time of surgery, it may appear norma externaIIy. However, the upper pouch wiI1 be seen to bulge when pressure is exerted on the anesthetic bag, thus confirming the atresia which has been demonstrated on roentgenograms.
SURGICAL
APPROACH
We prefer the extrapIeura1 over the intrapIeura1 approach to the posterior mediastinum for the surgica1 correction of these anomaIies. This approach is no more time-consuming, minimizes the anesthetic probIem and yieIds a quieter mediastinum during dissection and I97
CongenitaI
AnomaIies
of Esophagus genital esophageal webs, which are a form of atresia without fistula, usuaIIy require resection of the segment bearing the web and end-to-encl anastomosis. The use of a longitudinal incision in the esophagus through the web and transverse repair results in stricture formation at the site of the repair. OccasionaIIy the web will rupture spontaneously, permitting feedings through a narrow lumen. We discoverecl that this had happened in a two year old chiIt1 who swallowed a nickel which lodged in the midesophagus. When the coin was removed at esophagoscopy, the partialI\- obstructing web was seen. At a later esophagoscopy this web of an almost was cut, aIIowing restoration normal lumen. Anesthesia. Our present preference is etheroxygen anesthesia administereci with a tightfitting mask. Local anesthesia alone was used in some of our cases, but its advantages seem to have been more than outlveighed by the dificulties caused by a struggling infant. In our experience intratracheal intubation led to too many complications associated with subglottic edema and obstruction of the upper air passages, which in two instances necessitateci tracheostomv. Use of the extrapIeura1 approach lessens the need for positive pressure during anesthesia. Gastrostomy is a useful proceGastrostomy. dure in the pre- and postoperative management of congenital atresia. The simple Stamm type gastrostomy as described by Gross [7] is relatively safe to perform, is adequate for infant feeding and closes quickly when the tube is removed. In the infant with atresia without f%tuIa the gastrostomy may be used immediately for feeding purposes. Gastrostomy should be performed as a decompression measure in atresia with fistula in lvhich severe distention of the stomach and intestines causes respiratory embarrassment. The gastrostomy tube should be Ieft open or kept under gentle suction but shouId not be used for feeding purposes until the fistula is cIosed. This measure is especiaIIy vaIuabIe when transporting an infant to a distant city for definitive care. Gastrostomy may be performed immediateIy folIowing the surgical repair of the anomaIy if the infant’s condition seems satisfactory, or it may be performed as a separate procedure one or more days Iater. In patients having exceIIent anastomosis and being fed through a small pIastic tube, gastrostomy will not be necessary.
point of anastomosis. After these sutures have been tied, the excess amount of tissue is trimmed away from the lower segment. The muscuIature of the lower tip of the upper segment is then excised and the mucosal layer opened. Accurate closure of the mucosal Iayer is carried out using fine silk sutures on atrauthe knots into the matic@ needIes, inverting lumen. Before the final sutures are tied, a small pIastic tube (No. 190) may be passed down through a nostri1 into the pharynx and upper esophagus and on through the anastomosis into the stomach, as advised by Garrison and Mayer [g]. This tube wiI1 be used for feeding the infant in the immediate postoperative period. Following completion of the mucosal layer, interrupted sutures of the same material are used to approximate the muscuIar layer, avoiding mattress sutures since they tend to impair the blood suppIy of the ends of the segWhenever possible the anastomosis ments. should not be in juxtaposition to the repair of the fistuIa in the posterior wall of the trachea. If readily available, a Iayer of mediastinal fascia shouId be pIaced between the trachea and esophagus in an attempt to prevent recurrence of a tracheoesophageal fistula in the event of leakage at the site of anastomosis. If the esophagus is under any tension following the the upper segment should be anastomosis, secured to the fascia overlying the bodies of the vertebrae with fine sutures so as to relieve tension on the anastomosis when the infant swallows, as suggested by Swenson [6]. We have empIoyed drainage of the mediastinum using a smaI1 Penrose drain. Others have questioned this practice, but we beIieve it has value in giving daiI$ observation of the character of the drainage. The earliest indication of a Ieak is the appearance of saIiva and mucus on the dressing. This finding makes immediate gastrostomy mandatory. In genera1 the surgical approach for correction of tracheoesophagea1 fistula without atresia and atresia without fistuIa does not differ from that for atresia with fistula. Each anomaly presents its own technica problem. In the case of the fistula without atresia the major probIem is the separation of the esophagus from the trachea to which it may be fused for a distance of approximately I cm. above and beIow the fistula. This may make it necessary to transect the esophagus, close the fistuIa and perform an end-to-end anastomosis. Con‘99
Shaw and Pa&on Gastrostomy once instituted shouId be maintained even though not used unti1 it is evident that feeding by mouth wilI be satisfactory and that diIatation of a stricture at the site of the anastomosis wiI1 not be required. Administration of Fluid. Administration of ffuid wiI1 not be necessary during the first three or four days of Iife since the full term newborn is pIethoric. If, however, the diagnosis has been deIayed and the infant is dehydrated, fluids shouId be given subcutaneousIy. During the surgica1 procedure IO cc. of whoIe bIood per pound of body weight is given through a cannuIated ankIe vein. An additional IO cc. of cIear fluid per pound of body weight is given sIowIy at the conclusion of the operation if the infant is dehydrated. The cannuIa is then removed to eIiminate the danger of fluid overIoad, which is especiaIIy hazardous for the infant in respiratory distress. FolIowing surgery, daiIy fluids are limited to 50 cc. per pound of body weight for at Ieast three days. The fluids are given through the smaI1 indweIIing pIastic tube or the gastrostomy tube. Beginning with the fourth postoperative day the amount of fIuid is graduaIIy increased unti1 the chiId is receiving an adequate intake. A roentgenogram is made of the esophagus, using opaque media, on the eighth postoperative day if there has been no evidence of Ieakage from the anastomosis. Feedings by mouth, using an eye dropper as suggested by Potts [S], are begun if this examination reveaIs a we11 heaIed esophagus. Gastrostomy feedings are continued if a Ieak at the anastomotic site is demonstrated. Most of these Ieaks demonstrated at this time are smaI1 and wiI1 hea with the esophagus at rest and with adequate drainage of the mediastinum. Tracbeostomy. Tracheostomy may become necessary in the management of the infant having tracheoesophagea1 fistuIa because of obstruction of the upper air passages due to trauma from instrumentation, and to provide an avenue for aspiration of bronchia secretions to heIp cIear severe pneumonitis caused by aspirationa episodes. Care of the tracheostomy in the newborn is diffIcuIt and requires constant nursing attention. DecannuIation often must be deIayed. Complications. The earIy compIications are ateIectasis and pneumonitis due to the dua1 threat to the respiratory tract. Antimicrobial therapy empIoying peniciIIin and streptomycin 200
should be started immediateIy. AteIectasis of a singIe Iobe, usuaIIy the right upper Iobe, is not a serious compIication and often cIears during the operation. OverIoading the infant in respiratory distress with parentera fluids should be studiousIy avoided. Late compIications are almost aIways due to fauIts in heaIing at the site of anastomosis, nameIy, (I) esophagocutaneous f%tuIa, (2) recurrent tracheoesophagea1 fistuIa, and (3) stricture. The esophagocutaneous fistuIa wiI1 cIose if adequate drainage of the mediastinum is maintained. This compIication, however, does predispose to stricture formation. Recurrence of the tracheoesophagea1 fistuIa shouId be suspected when, foIIowing surgica1 repair, an infant coughs excessiveIy, especiaIIy when fed. This complication frequentIy is accompanied with a stricture at the anastomotic site and requires reoperation as soon as the mediastinitis has compIeteIy subsided. Some degree of stricture formation must be expected in a11 infants having repair of atresia of the esophagus. (Fig. 3.) In the earIy postoperative period no attempt should be made to diIate the stricture since it is very easy to tear the fragiIe tissue. Gastrostomy feeding shouId be continued and secretions coIIecting in the pharynx and upper esophagus aspirated. In many infants, as sweIIing at the anastomotic site subsides, an adequate Iumen wiI1 result. In others, when the esophagus is we11 heaIed, one or two carefu1 diIatations may be a11 that is required. We prefer to treat high grade strictures by reanastomosis foIIowing excision of the narrow esophageal segment. Substitution Procedures. AIthough primary anastomosis of the esophagea1 segments after division and cIosure of the fistula is the procedure of choice for atresia of the esophagus with tracheoesophagea1 fistula, this procedure is not appIicabIe in the absence of an adequate Iower esophagea1 segment or if the gap between the upper and Iower segments is too great to permit satisfactory anastomosis. In these patients substitution procedures, using either stomach or jejunum, are necessary to provide aIimentary continuity. The high operative mortality of an unsatisfactory end-to-end anastomosis may be reduced by division and closure of the fistuIa, cervical esophagostomy and gastrostomy at the first operation, to be foIIowed at a Iater date with a substitution procedure. Many different types of reconstructive procedures have been
CongenitaI
F~ti. 3A. Infant tracheocsophagca1
with atresia fistula.
of the
esophagus
AnomaIies
with
of Esophagus
FIG. 3B. Same infant ten days foIIowing cIosure of fist& with anastomosis of esophagus. Note narrowing at the site of anastomosis. This chiId now swaIIows a norma diet without difkulty.
described for the treatment of anomalies unsuitable for anastomosis. Gross [7] has suggested &ding the stomach and esophagus through a diIated hiatus in the diaphragm to facilitate an anastomosis between a short lower esophagea1 segment and the upper esophageal pouch. Byron [IO], Swenson [I I] and others have performed immediate transthoracic esophagogastrostomy in the newborn. The mortality of primary intrathoracic esophagogastrostomy has been high. In generaI, those who survived have faiIed to mature physicaIIy at a normal rate. Sweet [12], Haight [5] and Potts [8] have al1 emphasized the hazards of intrathoracic placement of the stomach in a newborn infant whose respiratory function has already been impaired by the anomaly itseIf. DeIayed procedures for restoration of alimentary continuity would appear to be prudent where primary anastomosis is impossible or unwise. The absence of air in the stomach or intestine is not sufficient indication to abandon extrapIeura1 mediastinal expIoration. Atresia of the
esophagus without the fIstuIa but with the Iower esophageal segment in muscuIar continuity with the upper pouch was found in one of our patients. Secondly, mediastinal exploration is necessary since it is possibIe to have a small fIstuIous opening which wouId otherwise not become apparent until feedings through the gastrostomy are attempted. AIimentary continuity may be provided by etevation of either stomach or jejunum to the neck for anastomosis to the esophagus. In generaI, most surgeons have pIaced the organ being substituted on the anterior chest wall outside the thorax. Sweet [ 121 successfuIIy transpIanted the stomach of a twenty-one month old chiId into the chest and anastomosed the fundus to the cervica1 esophagus. Rienhff [r~], Bigger [r4] and Potts [8] pIaced the entire stomach beneath the skin of the anterior chest wail and performed cervica1 esophagogastrostomy. Potts subsequently transplanted the stomach into 201
Shaw and Pa&on fistuIa if present, and gastrostomy were performed at the time of the first procedure. These children were fed by gastrostomy tube for nearIy two years, at which time a Roux-Y loop of jejunum was prepared and placed in a substerna1 tunnel for anastomosis with the proximal esophageal segment in the neck. The crux of the operation consists of careful preparation and straightening of the jejuna1 Ioop to be transplanted. After inspection and study of the jejunal mesenteric vessels, the serosa, fat, Iymphatics and nerves are carefuIIy stripped from these vessels we11 up onto the jejunum. In accordance with the vascuIar pattern, two or three principa1 jejunal vessels are cIamped, taking care to preserve an adequate bIood supply for the proxima1 iejunal segment just beIow the Iigament of Treitz. After determining the adequacy of the bIood suppIy and the desired Iength of the jejunal Ioop the appropriate principal vesseIs are divided and ligated. The jejunum is divided 6 to IO cm. beyond the Iigament of Treitz and the Iength of the resuIting jejuna1 segment is measured. Division of additiona main jejunal vesseIs may be necessary to provide suffxcient Iength to reach the neck. However, it is important to reaIize that the more jejuna1 vesseIs that are divided, the greater is the distance necessary to be bridged by the straightened jejuna1 segment. The substerna space is entered in the extraperitonea1 and extrafascia space, freeing the diaphragm wideIy from its attachments to the sternum and Costa1 margin. By bIunt dissection with the fingers the anterior mediastinal space is widened, pushing the pIeura to either side, progressing gently in an upward direction anterior to the pericardium. A cervica1 incision is made aIong the anterior border of the sternocIeidomastoid muscle and the proximal esophagus dissected free. The deep cervica1 fascia is incised and the sternothyroid and sternohyoid muscIes are divided. The substerna space is then entered from above and the tunnel widened to admit two or three fingers. By blunt dissection from above and beIow, the tunne1 is extended in both directions unti1 the fingers meet. Attention should be directed to the preparation of a wide tunnel to provide ampIe space for the transpIanted jejunum. The prepared Ioop of jejunum, having been previousIy temporariIy cIosed and the sutures
FIG. 4. Substernal trnnspkmtation of the jejunum and cervical esophagognstrostomy in a child in whom end-to-end annstomosis was impossible.
the left side of the chest because of the undesirable cosmetic appearance. Others, incIuding Davis and Stafford [IT], Ladd and Swenson [16] and Lam [z7], have used a segment of jejunum and a skin tube. Longmire [IS], Leven and Varco [zg], and others have used a limb of jejunum as an antethoracic transpIant simiIar to the method of Roux [zo] and Yudin [21]. CervicaI esophagojejunostomy is completed at a Iater stage. The disadvantages of an intrathoracic stomach in a chiId and the undesirable cosmetic appearance of an antethoracic stomach or jejunal Ioop make attractive intrathoracic, substerna placement of a Roux-Y jejuna1 Ioop as originaIIy suggested by Robertson and Sarjeant [22]. The substerna route constitutes the shortest distance to the neck and it is extrapIeura1, obviating the risks of an intrapleura1 pIacement. We have utiIized substerna pIacement of the jejunum and cervica1 esophagojejunostomy with satisfaction in three chiIdren in whom primary end-to-end anastomosis of the esophageaI segments was impossibIe or unsatisfactory. (Fig. 4.) In al1 patients cervica1 division and suture of the esophagostomy, 202
CongenitaI
AnomaIies
left Iong, is then brought through the transverse mesocoIon, anterior to the stomach and out of the peritonea1 cavity into the substerna space. It is both puIIed and gentIy pushed through the anterior mediastina1 substerna tunne1 to the neck. Great care is taken to avoid tension on bIood vessels and in genera1 to aIIow the jejunum to lie on its side, usuaIIy toward the right. If sufficient Iength of jejunum has been provided, end-to-end esophagojejunostomy is performed. The manubrium may be divicIed and anastomosis performed beneath it if necessary. After the completion of the cervical procedures end-to-end anastomosis of the proximal jejunum to the side of the straightened loop beIow the transverse mesocoIon is accompIished. The mesocolon is sutured to the jejunum and the abdominal incision c1osed about the bowel as it Ieaves the peritoneum. No attempt should be made to anastomose the stomach and transplanted Ioop of jejunum at this stage. These children usua11y require gastrostomy feeding for a variable period of time during the postoperative period and until such time as the chiId has become educated in ora feeding. At a later date side-to-side anastomosis of the jejunum to the antrum of the stomach may be easily accompIished to correct the by-pass of the stomach and its deIeterious effects on deveIopment.
of Esophagus OTHER
ANOMALIES
RedupIications of the esophagus and paraesophageal cysts may not be symptom producing. Often their presence is indicated by an abnormal shadow seen on a roentgenogram of the chest. Their surgical remova usuaI1y can be easiIy accomplished by emp1oying a transpIeural approach to the posterior mediastinum. Epiphrenic esophagea1 diverticula are considered by most authors to be acquired diverticula of the pu1sion type. One patient treated surgicaIIy by us for this type of diverticulum had one brother who a1so had a similar diverticuIum removed surgically and another brother who was the father of two infants, both born with atresia of the esophagus. Our experience with this family and the history often obtained of symptoms going back to early childhood lead us to beIieve that these diverticula may be congenital in origin. They are easily excised surgicaIIy using a transpIeura1 approach. CONCLUSION
Congenital anomaIies of the esophagus in which atresia or a fistuIous connection are present are urgent surgica1 problems. More than half of these infants can be saIvaged by direct end-to-end anastomosis of these two segments of the esophagus, or by deIayed substitution procedures when the former is not feasible. Associated anoma1ies incompatible with Iife, aspirational episodes causing puImonary infections and compIications due to faulty heaIing of the anastomosed esophagus are the greatest obstacles encountered in the surgica1 management of these infants. Other esophageal anomalies present surgica1 problems Iess urgent in nature and more easily managed.
RESULTS
The saIvage rate for atresia of the esophagus corrected surgicaIIy is steadiIy improving since the first successfu1 end-to-end anastomosis performed by Haight in 1940. Reports by Gross [7], Haight [f], Humphreys et a1. [I], Swenson [6], Potts [8] and others show a salvage rate ranging from 40 to 40 per cent of infants treated for this anomaIy since 1939. In the later years of this period the saIvage rate in many series of cases has reached as high as 80 per cent. We have twenty-seven Iiving children from a group of sixty-five babies (41.5 per cent) treated since 1938. During the past five years our resuIts have improved with the saIvage of twenty-two of thirty-four babies treated (65 per cent). Other associated anomaIies incompatible with Iife and the poor genera1 condition of many of these babies when presented for surgica1 therapy make it difficult to improve these resuIts appreciably.
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204
Congenital
DISEASES
Anomalies
ROBERT R. SHAW,
M.D.
AND DONALD L. PAULSON, M.D., Dallas, Texas
From tbe Department of Surgery of tbe University of Texas Soutbwestern Medical School and Baylor Hospital, Dallas, Texas.
ONGENITAL anomahes of the esophagus resuit from a fauIt in deveIopment at about the fourth week in the Iife of the embryo. A groove in the ventra1 waI1 of the primitive pharynx deepens and converts into the Iaryngotrachea1 tube. This tube is in intimate reIation to the upper part of the foregut, the Iengthening of which forms the esophagus. Incomplete separation of the trachea and esophagus, and faiIure of recanaIization of the esophagus resuIt in: (I) atresia of the esophagus with tracheoesophagea1 fistuIa, (2) atresia without tracheoesophagea1 f%tuIa, and (3) tracheoesophagea1 IistuIa without atresia. Other anomaIies of the esophagus are: (I) redupIications of the esophagus, (2) paraesophagea1 cysts, and (3) diverticuIa. Atresia of the esophagus with or without tracheoesophagea1 fistuIa is the most common anomaIy of the esophagus. Humphreys et aI. [I] report that it occurs in a ratio of approximateIy I :3,000 births. Its surgica1 correction is a diI%cuIt task, which taxes the ski11 and experience of the surgeon, anesthetist and hospita1 staff invoIved in the postoperative care. The reader is referred to exceIIent contributions that have been made by Lanman [2], Ladd and Gross [?I, Leven [4], Haight [5], Swenson [6], Gross [7], Humphreys et a1. [I], Potts [8] and others. UnIess corrected surgicahy, the anomaIy is incompatible with life since the infant cannot be fed. The earIy threat, however, is chieAy to the respiratory tract from aspiration of secretions from the mouth and reffux of the gastrointestina1 secretions through the Iower esopha-
C
American
Journal of Surgery. Volume 93. February.
of the Esophagus
1977
gus into the trachea. This latter danger is the greater one. During the period before the anomaIy is corrected the infant shouId be pIaced in a semi-upright position to aIIow gravity to overcome the tendency toward retIux. Nursing attendants shouId be vigilant in the aspiration of pharyngea1 secretions. There shouId be no immediate concern about the giving of parentera fIuids, since the newborn infant at term is pIethoric and we11 abIe to endure three or four days without fluids. DIAGNOSIS Atresia of the esophagus shouId be suspected when a newborn infant drooIs excessiveIy, chokes upon feedings and has cyanotic episodes. The diagnosis can be confirmed by the inabiIity to pass a catheter into the stomach and the VisuaIization of a bIind upper pouch on roentgenogram after putting opaque media (never barium) into the upper esophagus. The presence of air in the stomach and intestines as shown by the roentgenogram establishes the fact that a tracheoesophagea1 fIstuIa is present. The absence of air suggests that there is no fistula between the Iower esophagus and trachea but does not ruIe out this possibihty. Tracheoesophagea1 IistuIa without atresia shouId be suspected when a newborn infant has severe coughing episodes when fed. The fistuIa may be demonstrated by roentgenograms taken after opaque media has been pIaced in the esophagus with the infant in the prone position. ANATOMY OF
THE
ANOMALIES
Atresia of the esophagus with tracheoesophagea1 fistuIa, in which the Iower esophagus enters the posterior waI1 of the trachea, makes 196
CongenitaI
AnomaIies
FIG. IA. This roentgrnogram shows the typical anatomy of congenital atresia of the esophagus with tracheoesophagea1 fistula. Note smaIl tapered Iower segment communicating with trachea. (It is not advisabIe to use this amount of opaque media during the roentgenographic examination.)
of Esophagus
Flc;. IB. This is the same type of anomaly as shown in Figure IA. Note Iong upper blind pouch overIapping large Iower scgmcnt which forms a fistuta with the trachea.
Atresia of the esophagus without tracheoesophagea1 fistuIa is variabIe in the Iength of the upper esophagea1 pouch. (Fig. 2.) The Iower esophagus may vary from an atretic cord of tissue to a sharpIy tapering esophagus ending bIindIy in the posterior mediastinum adherent to but not communicating with the trachea. A variant of this anomaly is the congenita1 esophagea1 web Iocated at the mid-esophagus. TracheoesophageaI fistuIa without atresia is represented by a we11 developed esophagus which is intimateIy adherent to the trachealis muscle aIong the lower portion of the trachea with a fistuIous opening above the bifurcation. In rare instances two fistuIas may be present, with the upper segment as we11 as the Iower segment communicating with the trachea.
up 90 per cent of the anomaIies of this type. There is considerabIe anatomic variation in this anomaly. The upper esophagus, which is a blind pouch, is uniformIy thickened but may vary in Iength from a smaI1 pouch high in the thorax to a bIind segment extending aImost to the diaphragm. (Fig. I.) In Iike manner, the lower esophagus may be we11 developed and enter the posterior trachea1 waI1 with a patuIous opening as high as 2 cm. above the bifurcation of the trachea, or it may be a smaI1 underdeveIoped muscuIar tube which tapers sharpIy at its upper end to form a small fistuIa at the bifurcation of the trachea or at the upper portion of the Ieft main bronchus. Thus the two esophagea1 segments may overIap 2 to 3 cm. or there may be a gap of similar Iength between their two ends. When an overIapping esophagus is exposed at the time of surgery, it may appear norma externaIIy. However, the upper pouch wiI1 be seen to bulge when pressure is exerted on the anesthetic bag, thus confirming the atresia which has been demonstrated on roentgenograms.
SURGICAL
APPROACH
We prefer the extrapIeura1 over the intrapIeura1 approach to the posterior mediastinum for the surgica1 correction of these anomaIies. This approach is no more time-consuming, minimizes the anesthetic probIem and yieIds a quieter mediastinum during dissection and I97
CongenitaI
AnomaIies
of Esophagus genital esophageal webs, which are a form of atresia without fistula, usuaIIy require resection of the segment bearing the web and end-to-encl anastomosis. The use of a longitudinal incision in the esophagus through the web and transverse repair results in stricture formation at the site of the repair. OccasionaIIy the web will rupture spontaneously, permitting feedings through a narrow lumen. We discoverecl that this had happened in a two year old chiIt1 who swallowed a nickel which lodged in the midesophagus. When the coin was removed at esophagoscopy, the partialI\- obstructing web was seen. At a later esophagoscopy this web of an almost was cut, aIIowing restoration normal lumen. Anesthesia. Our present preference is etheroxygen anesthesia administereci with a tightfitting mask. Local anesthesia alone was used in some of our cases, but its advantages seem to have been more than outlveighed by the dificulties caused by a struggling infant. In our experience intratracheal intubation led to too many complications associated with subglottic edema and obstruction of the upper air passages, which in two instances necessitateci tracheostomv. Use of the extrapIeura1 approach lessens the need for positive pressure during anesthesia. Gastrostomy is a useful proceGastrostomy. dure in the pre- and postoperative management of congenital atresia. The simple Stamm type gastrostomy as described by Gross [7] is relatively safe to perform, is adequate for infant feeding and closes quickly when the tube is removed. In the infant with atresia without f%tuIa the gastrostomy may be used immediately for feeding purposes. Gastrostomy should be performed as a decompression measure in atresia with fistula in lvhich severe distention of the stomach and intestines causes respiratory embarrassment. The gastrostomy tube should be Ieft open or kept under gentle suction but shouId not be used for feeding purposes until the fistula is cIosed. This measure is especiaIIy vaIuabIe when transporting an infant to a distant city for definitive care. Gastrostomy may be performed immediateIy folIowing the surgical repair of the anomaIy if the infant’s condition seems satisfactory, or it may be performed as a separate procedure one or more days Iater. In patients having exceIIent anastomosis and being fed through a small pIastic tube, gastrostomy will not be necessary.
point of anastomosis. After these sutures have been tied, the excess amount of tissue is trimmed away from the lower segment. The muscuIature of the lower tip of the upper segment is then excised and the mucosal layer opened. Accurate closure of the mucosal Iayer is carried out using fine silk sutures on atrauthe knots into the matic@ needIes, inverting lumen. Before the final sutures are tied, a small pIastic tube (No. 190) may be passed down through a nostri1 into the pharynx and upper esophagus and on through the anastomosis into the stomach, as advised by Garrison and Mayer [g]. This tube wiI1 be used for feeding the infant in the immediate postoperative period. Following completion of the mucosal layer, interrupted sutures of the same material are used to approximate the muscuIar layer, avoiding mattress sutures since they tend to impair the blood suppIy of the ends of the segWhenever possible the anastomosis ments. should not be in juxtaposition to the repair of the fistuIa in the posterior wall of the trachea. If readily available, a Iayer of mediastinal fascia shouId be pIaced between the trachea and esophagus in an attempt to prevent recurrence of a tracheoesophageal fistula in the event of leakage at the site of anastomosis. If the esophagus is under any tension following the the upper segment should be anastomosis, secured to the fascia overlying the bodies of the vertebrae with fine sutures so as to relieve tension on the anastomosis when the infant swallows, as suggested by Swenson [6]. We have empIoyed drainage of the mediastinum using a smaI1 Penrose drain. Others have questioned this practice, but we beIieve it has value in giving daiI$ observation of the character of the drainage. The earliest indication of a Ieak is the appearance of saIiva and mucus on the dressing. This finding makes immediate gastrostomy mandatory. In genera1 the surgical approach for correction of tracheoesophagea1 fistula without atresia and atresia without fistuIa does not differ from that for atresia with fistula. Each anomaly presents its own technica problem. In the case of the fistula without atresia the major probIem is the separation of the esophagus from the trachea to which it may be fused for a distance of approximately I cm. above and beIow the fistula. This may make it necessary to transect the esophagus, close the fistuIa and perform an end-to-end anastomosis. Con‘99
Shaw and Pa&on Gastrostomy once instituted shouId be maintained even though not used unti1 it is evident that feeding by mouth wilI be satisfactory and that diIatation of a stricture at the site of the anastomosis wiI1 not be required. Administration of Fluid. Administration of ffuid wiI1 not be necessary during the first three or four days of Iife since the full term newborn is pIethoric. If, however, the diagnosis has been deIayed and the infant is dehydrated, fluids shouId be given subcutaneousIy. During the surgica1 procedure IO cc. of whoIe bIood per pound of body weight is given through a cannuIated ankIe vein. An additional IO cc. of cIear fluid per pound of body weight is given sIowIy at the conclusion of the operation if the infant is dehydrated. The cannuIa is then removed to eIiminate the danger of fluid overIoad, which is especiaIIy hazardous for the infant in respiratory distress. FolIowing surgery, daiIy fluids are limited to 50 cc. per pound of body weight for at Ieast three days. The fluids are given through the smaI1 indweIIing pIastic tube or the gastrostomy tube. Beginning with the fourth postoperative day the amount of fIuid is graduaIIy increased unti1 the chiId is receiving an adequate intake. A roentgenogram is made of the esophagus, using opaque media, on the eighth postoperative day if there has been no evidence of Ieakage from the anastomosis. Feedings by mouth, using an eye dropper as suggested by Potts [S], are begun if this examination reveaIs a we11 heaIed esophagus. Gastrostomy feedings are continued if a Ieak at the anastomotic site is demonstrated. Most of these Ieaks demonstrated at this time are smaI1 and wiI1 hea with the esophagus at rest and with adequate drainage of the mediastinum. Tracbeostomy. Tracheostomy may become necessary in the management of the infant having tracheoesophagea1 fistuIa because of obstruction of the upper air passages due to trauma from instrumentation, and to provide an avenue for aspiration of bronchia secretions to heIp cIear severe pneumonitis caused by aspirationa episodes. Care of the tracheostomy in the newborn is diffIcuIt and requires constant nursing attention. DecannuIation often must be deIayed. Complications. The earIy compIications are ateIectasis and pneumonitis due to the dua1 threat to the respiratory tract. Antimicrobial therapy empIoying peniciIIin and streptomycin 200
should be started immediateIy. AteIectasis of a singIe Iobe, usuaIIy the right upper Iobe, is not a serious compIication and often cIears during the operation. OverIoading the infant in respiratory distress with parentera fluids should be studiousIy avoided. Late compIications are almost aIways due to fauIts in heaIing at the site of anastomosis, nameIy, (I) esophagocutaneous f%tuIa, (2) recurrent tracheoesophagea1 fistuIa, and (3) stricture. The esophagocutaneous fistuIa wiI1 cIose if adequate drainage of the mediastinum is maintained. This compIication, however, does predispose to stricture formation. Recurrence of the tracheoesophagea1 fistuIa shouId be suspected when, foIIowing surgica1 repair, an infant coughs excessiveIy, especiaIIy when fed. This complication frequentIy is accompanied with a stricture at the anastomotic site and requires reoperation as soon as the mediastinitis has compIeteIy subsided. Some degree of stricture formation must be expected in a11 infants having repair of atresia of the esophagus. (Fig. 3.) In the earIy postoperative period no attempt should be made to diIate the stricture since it is very easy to tear the fragiIe tissue. Gastrostomy feeding shouId be continued and secretions coIIecting in the pharynx and upper esophagus aspirated. In many infants, as sweIIing at the anastomotic site subsides, an adequate Iumen wiI1 result. In others, when the esophagus is we11 heaIed, one or two carefu1 diIatations may be a11 that is required. We prefer to treat high grade strictures by reanastomosis foIIowing excision of the narrow esophageal segment. Substitution Procedures. AIthough primary anastomosis of the esophagea1 segments after division and cIosure of the fistula is the procedure of choice for atresia of the esophagus with tracheoesophagea1 fistula, this procedure is not appIicabIe in the absence of an adequate Iower esophagea1 segment or if the gap between the upper and Iower segments is too great to permit satisfactory anastomosis. In these patients substitution procedures, using either stomach or jejunum, are necessary to provide aIimentary continuity. The high operative mortality of an unsatisfactory end-to-end anastomosis may be reduced by division and closure of the fistuIa, cervical esophagostomy and gastrostomy at the first operation, to be foIIowed at a Iater date with a substitution procedure. Many different types of reconstructive procedures have been
CongenitaI
F~ti. 3A. Infant tracheocsophagca1
with atresia fistula.
of the
esophagus
AnomaIies
with
of Esophagus
FIG. 3B. Same infant ten days foIIowing cIosure of fist& with anastomosis of esophagus. Note narrowing at the site of anastomosis. This chiId now swaIIows a norma diet without difkulty.
described for the treatment of anomalies unsuitable for anastomosis. Gross [7] has suggested &ding the stomach and esophagus through a diIated hiatus in the diaphragm to facilitate an anastomosis between a short lower esophagea1 segment and the upper esophageal pouch. Byron [IO], Swenson [I I] and others have performed immediate transthoracic esophagogastrostomy in the newborn. The mortality of primary intrathoracic esophagogastrostomy has been high. In generaI, those who survived have faiIed to mature physicaIIy at a normal rate. Sweet [12], Haight [5] and Potts [8] have al1 emphasized the hazards of intrathoracic placement of the stomach in a newborn infant whose respiratory function has already been impaired by the anomaly itseIf. DeIayed procedures for restoration of alimentary continuity would appear to be prudent where primary anastomosis is impossible or unwise. The absence of air in the stomach or intestine is not sufficient indication to abandon extrapIeura1 mediastinal expIoration. Atresia of the
esophagus without the fIstuIa but with the Iower esophageal segment in muscuIar continuity with the upper pouch was found in one of our patients. Secondly, mediastinal exploration is necessary since it is possibIe to have a small fIstuIous opening which wouId otherwise not become apparent until feedings through the gastrostomy are attempted. AIimentary continuity may be provided by etevation of either stomach or jejunum to the neck for anastomosis to the esophagus. In generaI, most surgeons have pIaced the organ being substituted on the anterior chest wall outside the thorax. Sweet [ 121 successfuIIy transpIanted the stomach of a twenty-one month old chiId into the chest and anastomosed the fundus to the cervica1 esophagus. Rienhff [r~], Bigger [r4] and Potts [8] pIaced the entire stomach beneath the skin of the anterior chest wail and performed cervica1 esophagogastrostomy. Potts subsequently transplanted the stomach into 201
Shaw and Pa&on fistuIa if present, and gastrostomy were performed at the time of the first procedure. These children were fed by gastrostomy tube for nearIy two years, at which time a Roux-Y loop of jejunum was prepared and placed in a substerna1 tunnel for anastomosis with the proximal esophageal segment in the neck. The crux of the operation consists of careful preparation and straightening of the jejuna1 Ioop to be transplanted. After inspection and study of the jejunal mesenteric vessels, the serosa, fat, Iymphatics and nerves are carefuIIy stripped from these vessels we11 up onto the jejunum. In accordance with the vascuIar pattern, two or three principa1 jejunal vessels are cIamped, taking care to preserve an adequate bIood supply for the proxima1 iejunal segment just beIow the Iigament of Treitz. After determining the adequacy of the bIood suppIy and the desired Iength of the jejunal Ioop the appropriate principal vesseIs are divided and ligated. The jejunum is divided 6 to IO cm. beyond the Iigament of Treitz and the Iength of the resuIting jejuna1 segment is measured. Division of additiona main jejunal vesseIs may be necessary to provide suffxcient Iength to reach the neck. However, it is important to reaIize that the more jejuna1 vesseIs that are divided, the greater is the distance necessary to be bridged by the straightened jejuna1 segment. The substerna space is entered in the extraperitonea1 and extrafascia space, freeing the diaphragm wideIy from its attachments to the sternum and Costa1 margin. By bIunt dissection with the fingers the anterior mediastinal space is widened, pushing the pIeura to either side, progressing gently in an upward direction anterior to the pericardium. A cervica1 incision is made aIong the anterior border of the sternocIeidomastoid muscle and the proximal esophagus dissected free. The deep cervica1 fascia is incised and the sternothyroid and sternohyoid muscIes are divided. The substerna space is then entered from above and the tunnel widened to admit two or three fingers. By blunt dissection from above and beIow, the tunne1 is extended in both directions unti1 the fingers meet. Attention should be directed to the preparation of a wide tunnel to provide ampIe space for the transpIanted jejunum. The prepared Ioop of jejunum, having been previousIy temporariIy cIosed and the sutures
FIG. 4. Substernal trnnspkmtation of the jejunum and cervical esophagognstrostomy in a child in whom end-to-end annstomosis was impossible.
the left side of the chest because of the undesirable cosmetic appearance. Others, incIuding Davis and Stafford [IT], Ladd and Swenson [16] and Lam [z7], have used a segment of jejunum and a skin tube. Longmire [IS], Leven and Varco [zg], and others have used a limb of jejunum as an antethoracic transpIant simiIar to the method of Roux [zo] and Yudin [21]. CervicaI esophagojejunostomy is completed at a Iater stage. The disadvantages of an intrathoracic stomach in a chiId and the undesirable cosmetic appearance of an antethoracic stomach or jejunal Ioop make attractive intrathoracic, substerna placement of a Roux-Y jejuna1 Ioop as originaIIy suggested by Robertson and Sarjeant [22]. The substerna route constitutes the shortest distance to the neck and it is extrapIeura1, obviating the risks of an intrapleura1 pIacement. We have utiIized substerna pIacement of the jejunum and cervica1 esophagojejunostomy with satisfaction in three chiIdren in whom primary end-to-end anastomosis of the esophageaI segments was impossibIe or unsatisfactory. (Fig. 4.) In al1 patients cervica1 division and suture of the esophagostomy, 202
CongenitaI
AnomaIies
left Iong, is then brought through the transverse mesocoIon, anterior to the stomach and out of the peritonea1 cavity into the substerna space. It is both puIIed and gentIy pushed through the anterior mediastina1 substerna tunne1 to the neck. Great care is taken to avoid tension on bIood vessels and in genera1 to aIIow the jejunum to lie on its side, usuaIIy toward the right. If sufficient Iength of jejunum has been provided, end-to-end esophagojejunostomy is performed. The manubrium may be divicIed and anastomosis performed beneath it if necessary. After the completion of the cervical procedures end-to-end anastomosis of the proximal jejunum to the side of the straightened loop beIow the transverse mesocoIon is accompIished. The mesocolon is sutured to the jejunum and the abdominal incision c1osed about the bowel as it Ieaves the peritoneum. No attempt should be made to anastomose the stomach and transplanted Ioop of jejunum at this stage. These children usua11y require gastrostomy feeding for a variable period of time during the postoperative period and until such time as the chiId has become educated in ora feeding. At a later date side-to-side anastomosis of the jejunum to the antrum of the stomach may be easily accompIished to correct the by-pass of the stomach and its deIeterious effects on deveIopment.
of Esophagus OTHER
ANOMALIES
RedupIications of the esophagus and paraesophageal cysts may not be symptom producing. Often their presence is indicated by an abnormal shadow seen on a roentgenogram of the chest. Their surgical remova usuaI1y can be easiIy accomplished by emp1oying a transpIeural approach to the posterior mediastinum. Epiphrenic esophagea1 diverticula are considered by most authors to be acquired diverticula of the pu1sion type. One patient treated surgicaIIy by us for this type of diverticulum had one brother who a1so had a similar diverticuIum removed surgically and another brother who was the father of two infants, both born with atresia of the esophagus. Our experience with this family and the history often obtained of symptoms going back to early childhood lead us to beIieve that these diverticula may be congenital in origin. They are easily excised surgicaIIy using a transpIeura1 approach. CONCLUSION
Congenital anomaIies of the esophagus in which atresia or a fistuIous connection are present are urgent surgica1 problems. More than half of these infants can be saIvaged by direct end-to-end anastomosis of these two segments of the esophagus, or by deIayed substitution procedures when the former is not feasible. Associated anoma1ies incompatible with Iife, aspirational episodes causing puImonary infections and compIications due to faulty heaIing of the anastomosed esophagus are the greatest obstacles encountered in the surgica1 management of these infants. Other esophageal anomalies present surgica1 problems Iess urgent in nature and more easily managed.
RESULTS
The saIvage rate for atresia of the esophagus corrected surgicaIIy is steadiIy improving since the first successfu1 end-to-end anastomosis performed by Haight in 1940. Reports by Gross [7], Haight [f], Humphreys et a1. [I], Swenson [6], Potts [8] and others show a salvage rate ranging from 40 to 40 per cent of infants treated for this anomaIy since 1939. In the later years of this period the saIvage rate in many series of cases has reached as high as 80 per cent. We have twenty-seven Iiving children from a group of sixty-five babies (41.5 per cent) treated since 1938. During the past five years our resuIts have improved with the saIvage of twenty-two of thirty-four babies treated (65 per cent). Other associated anomaIies incompatible with Iife and the poor genera1 condition of many of these babies when presented for surgica1 therapy make it difficult to improve these resuIts appreciably.
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