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Atresia Esofagus
Atresia Esofagus
Atresia Esofagus
A B C D E F
FIGURE 69-2 Gross classification of anatomic patterns of esophageal atresia. A, Esophageal atresia without a tracheoesophageal fistula. This malforma-
tion is almost invariably associated with a “long gap.” B, Atresia with a proximal tracheoesophageal fistula. It is an uncommon anomaly; the abdomen is
airless, and the diagnosis may be missed unless contrast studies or endoscopy is performed. C, Esophageal atresia with a distal tracheoesophageal fistula,
the most frequently encountered form of esophageal anomaly. D, Atresia with a double (proximal and distal) fistula. Though rare, this form is found more
often than originally thought. E, Tracheoesophageal fistula without atresia (H-type fistula). This anomaly may be missed in the newborn period because
swallowing is possible. It is associated with recurrent cough, pneumonia, and abdominal distention. F, Esophageal stenosis. (From Gross RE: Surgery of
Infancy and Childhood. Philadelphia, WB Saunders, 1953.)
TABLE 69-1 of the foregut was the result of fusion of invaginating lateral
Waterston Risk Groups and Current Survival Figures longitudinal ridges that created a septum dividing the foregut
into a dorsal digestive tract and a ventral respiratory system.
Group Survival (%) Waterston Classification
Formation of the so-called tracheoesophageal septum is
A 100 Birth weight > 2500 g and otherwise healthy believed to begin caudally and end cranially.7 Many of the
B 85 Birth weight 2000-2500 g and well or higher theories concerning the pathogenesis of EA malformations
weight with moderate associated anomalies have been based on this description.7,28–31 However, several
(noncardiac anomalies plus patent ductus
arteriosus, ventricular septal defect, and investigators have more recently found little evidence to
atrial septal defect) support this theory of normal foregut separation and develop-
C 65 Birth weight < 2000 g or higher with severe ment. Reinvestigation of human specimens from the Col-
associated cardiac anomalies lection of Embryos at the Carnegie Institute suggests that
ingrowth of lateral foregut wall ridges and cranial develop-
ment of a tracheoesophageal septum do not occur in the
In addition to anatomic classifications, in 1962 D. J. Waterston, human embryo.32,33 In addition, Kluth and colleagues34 stud-
R. E. Bonham Carter, and Eoin Aberdeen of the Hospital for ied staged chicken embryos via scanning electron microscopy
Sick Children in London developed a classification system rela- in an attempt to clarify normal foregut development. In this
ted to risk factors in infants with EA.26 This risk stratification model they also found no evidence of lateral ridges or an
allowed comparison of case outcomes over time and between epithelial tracheoesophageal septum.
hospitals. The use of this classification scheme—which was A summary of the major theories to explain the embryo-
based on birth weight, the presence of pneumonia, and associ- pathology of the foregut has recently been presented by Kluth
ated congenital anomalies—allowed the identification of factors and Fiegel.35 Theories of foregut occlusion and failure of
that predicted survival and guided operative treatment. This recanalization of the intestinal lumen have been histori-
approach to the classification of EA anomalies has been an cally prominent in discussions regarding the pathogenesis
important contribution to the care of these infants (Table 69-1). of intestinal atresias including EA.36,37
Overwhelming proliferation of esophageal epithelium may
play a role in the development of rare membranous EA, but it
Embryology falls short as a comprehensive theory of typical EA malforma-
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tions.25 Another theory has been put forth by Cozzi and col-
The pathogenesis of congenital EA malformations remains leagues38,39 and others40 in which it is suggested that EA is a
unknown. Although many theories have been offered to component of cephalic neurocristopathy. The observation that
explain the etiology of EA, EA-TEF, and TEF, no single unifying there is a clear association of neural crest-implicated cardio-
theory has been proposed that addresses all the variations seen vascular anomalies (aortic arch, conotruncal and membranous
with this group of anomalies. The pathogenesis is heteroge- ventricular septal defects), as well as thymic, thyroid, parathy-
neous and multifactorial and involves multiple genes and roid, and facial malformations with EA, as seen with the
complex gene-environment interactions. In addition, about DiGeorge syndrome, suggests that the pathogenesis of EA
half of the cases of EA are isolated and the other half are may be related to defective pharyngeal arch development.
associated with other malformations. An animal model in which Adriamycin is used as a terato-
A clear understanding of the pathogenesis of the EA gen and results in embryos with a variety of malformations,
malformation is hindered by the fact that normal foregut including EA and TEF, has been described and promises to
embryology is still obscure. Wilhelm His, Sr., the founder provide new insight into EA pathogenesis.41,42 Merei and
of human embryology, was the first to describe normal devel- Hutson have used this model to develop a theory suggesting
opment of the respiratory system.27 He believed that division that the primitive foregut develops into the trachea, with
896 PART VI THORAX
subsequent EA-TEF anomalies.43 Gittes has recently used this in the United States55 and Australia.56 According to data from
model, as well as human TEF tissue samples, to investigate the the California Birth Defects Monitoring Program database, from
molecular mechanisms that may play a role in the patho- 1983 to 1988 the total prevalence of EA, EA-TEF, and TEF alone
genesis of EA-TEF. He has proposed that the distal fistula tract was reported to be 2.82 per 10,000 live births and stillbirths.57
and esophagus are of respiratory origin and that defects in In Europe, the prevalence rate of EA-TEF during 1980 to 1988
lung morphogenesis account for this aspect of the EA-TEF was 2.86 per 10,000 births, although this rate appeared to be
anomaly.44,45 Insight into the pathogenesis of EA-TEF will also decreasing over time.58 In this European study, 62% of infants
come from a clearer understanding of the genetics of this and with EA-TEF were male, whereas the California database found
associated conditions. More than 50% of cases of EA-TEF are that male-to-female ratios varied considerably between types
associated with other anomalies, and up to 10% of cases are of EA-TEF defects, with ratios of 2.29 for TEF alone, 1.44
found in specific chromosomal or single-gene disorders. for EA-TEF, and 1.33 for EA alone.57 Mothers of white ethnicity
Chromosomal abnormalities associated with EA-TEF include have a higher (>60%) prevalence of EA-TEF and TEF than
DiGeorge syndrome; full trisomies (21, 13, and 18); Opitz nonwhite populations do.59,60 An increased risk for EA-TEF
syndrome; and 13q, 17q, and 16q24 deletions.46 Single-gene with a first pregnancy,59 as well as increasing maternal age,57
mutation examples include MYCN (Feingold syndrome), have been reported (twofold increased risk for women 35
CHD7 (CHARGE syndrome), SOX2 (AEG syndrome), GL13 to 40 years old and threefold increase for those older than
(Pallister-Hall syndrome), MID1 (Opitz G syndrome), and age 40).60
FANCA (Fanconia anemia).46 The rate of multiple births is high for each type of EA-TEF
Recent insights into the genetics involved in VACTERL asso- anomaly (TEF, 3.7%; EA-TEF, 4.9%; and EA alone, 8.8% in
ciation will also provide new information into the etiology of a control population).57 In addition, EA, along with other con-
EA-TEF. For example, the VACTERL-hydrocephalus association genital anomalies, is significantly increased in the offspring of
is related to mutations in the following genes: FANCC, in vitro fertilization patients (OR 3.65:CI 2/53-5/26).61
FANCD1 and 2, FACCG, FANCB, and PTEN. In addition to
VACTERL associations, EA-TEF is also increased in several
other conditions such as Goldenhar syndrome and Martinez- Associated Anomalies
Frias syndrome.47 The development of rat and mouse models ------------------------------------------------------------------------------------------------------------------------------------------------
of EA has recently allowed the parallel study of both normal The early disturbance in organogenesis that results in EA
and abnormal embryogenesis. As discussed earlier, contro- deformities, whatever the exact cause, also affects other organ
versies persist, but the fundamental morphogenetic process systems. Other congenital anomalies are frequently associated
appears to be a rearrangement of the proximal foregut into sep- with EA, and the associated anomaly often significantly alters
arate respiratory (ventral) and gastrointestinal (dorsal) tubes. treatment and affects survival. Numerous reports have sug-
This process depends on the precise temporal and spatial gested that between 50% and 70% of infants with EA have
pattern of expression of a number of foregut patterning at least one associated congenital malformation. The anoma-
genes. Disturbance of this pattern disrupts foregut separa- lies are most common in cases of EA without TEF and are
tion and underlies the development of tracheoesophageal least common in cases of H-type TEF.62 About half of all
malformations.48 Specific murine models of EA-TEF have sug- EA-TEF-associated malformations can be classified as part
gested a number of important genes related to pathogenesis of a recognizable malformation syndrome such as chromo-
that are mostly in developmental pathways including vitamin somal, VACTERL, CHARGE, Fanconi anemia, Opitz G, and
A effectors, retinoic acid receptors a and b (Rara and Rarb), Goldenhar. The other 50% of patients with associated malfor-
sonic hedgehog pathway effectors (Shh, Gli2, Gli3, Foxf1), mations are considered as “nonsyndromic” with multiple
and other homeobox containing transcription factors (Hoxc4, congenital anomalies. In a recent study from France, these
Ttf-1, Pcsk5).46 As suggested by the Adriamycin rat model malformations included cardiovascular system (24%), uro-
for EA-TEF, environmental teratogens have also been implicated genital (21%), digestive (21%), musculoskeletal (14%), and
in the pathogenesis of EA ! TEF. EA has occurred in infants central nervous system (7%).63
born to mothers with prolonged exposure to contraceptive Considering reports over recent decades, cardiovascular
pills49 and exposure to progesterone and estrogen during a anomalies occur most frequently (11% to 49%),39,64–66 followed
pregnancy.50 In addition, EA has been reported in some infants by genitourinary (24%),67 gastrointestinal (24%),68 and muscu-
of hyperthyroid51 and diabetic mothers and after intrauterine loskeletal (13% to 22%)69,70 anomalies (Table 69-2). Associated
exposure to thalidomide and diethylstibestrol.46,52 neurologic anomalies include neural tube defects (2.3%), hydro-
Many studies have described transverse and vertical familial cephalus (5.2%), holoprosencephaly (2.3%), and anophthalmia
cases of all varieties of EA. Empirical risk figures based on the or microphthalmos (3.7%).59 Other reported associated anom-
literature to date give a 0.5% to 2% recurrent risk in siblings of alies include choanal atresia (5.2%), facial cleft (7.2%), abdom-
one affected child; the risk increases to 20% if more than one inal wall defects (4.3%), and diaphragmatic hernia (2.9%).59
sibling is affected.53 The empiric risk for a child born to an One report suggests that the incidence of certain associated
affected parent is 3% to 4%.53 anomalies may vary by world region in that genitourinary anom-
alies were found in 26% of a European cohort of patients versus
only 4% of an Asian cohort.70
Epidemiology
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Complex cardiac deformities may account for most of
the deaths associated with EA malformations. The relative risk
The reported incidence of EA, with or without TEF (EA ! for death in infants with EA malformations associated with a
TEF), is 1:3500 live-born infants; however, it varies geograph- major cardiac anomaly is reported to be 3.47 (95% confidence
ically from 1 in 2440 births in Finland54 to 1 in 4500 births interval [CI], 1.51 to 7.96).65,71 In a series of 153 patients
CHAPTER 69 CONGENITAL ANOMALIES OF THE ESOPHAGUS 897
TABLE 69-2 a high mortality rate. Iuchtman and colleagues81 analyzed the
Incidence of Associated Anomalies results of 46 infants born with EA and features of the VAC-
Cardiovascular "24%
TERL association among 313 patients with EA, TEF, or both.
Genitourinary "21%
In this series, the mortality rate was 24%. Cardiovascular
Gastrointestinal "21%
anomalies were present in 78% of the patients and were the
Musculoskeletal "14%
principal cause of death. More recently, Driver and colleagues
Central nervous system "7%
reported that the relative risk for mortality in patients with
VACTERL association "20%
VACTERL associations was 2.54 (95% CI, 1.14 to 4.86).65
Overall incidence 50%-70%
EA malformations are increasingly being described in asso-
ciation with other polytopic defects, sequences, syndromes,
VACTERL, vertebral, anorectal, cardiac, tracheoesophageal, renal, and limb and associations. Some of these include Down syndrome82;
abnormalities. the DiGeorge sequence83; the polysplenia sequence; Holt-Oram
syndrome; the Pierre Robin sequence53; Feingold syndrome84;
with EA, Leonard and colleagues reported that the 1-year Fanconi syndrome; Townes-Brock syndrome; Bartsocas-Papas
survival rate of infants with congenital heart disease (CHD) syndrome; McKusick-Kaufman syndrome53,85–88; the CHARGE
was 67% versus 95% in those without CHD.66 Interestingly, association (coloboma, heart defects, atresia choanae, develop-
these authors concluded that although CHD is associated with mental retardation, genital hypoplasia, and ear deformities)89;
higher mortality in EA patients, it is not the direct cause of it.66 and the schisis association (omphalocele, neural tube defects,
The most common single defects identified are ventricular cleft lip and palate, and genital hypoplasia).90,91 Other serious
septal defects (19%), which have reportedly been associated or lethal associations are trisomy 18, cerebral hypoplasia, and
with a mortality rate of 16%72 and atrial septal defects Potter syndrome (bilateral renal agenesis).92,93 In addition to
(20%).64 Other common cardiac anomalies include the tetral- the well-described anomalies associated with EA just discussed,
ogy of Fallot (5%) and patent ductus arteriosus (13%).64 many recent reports describe significant malformations and
Coarctation of the aorta is found in 1% to 4% of infants with associated conditions that may become more frequently recog-
EA malformations.64,73 A descending aorta on the right side nized. Unilateral pulmonary agenesis associated with EA-TEF
has been reported in 4% of infants with EA.64 is a rare condition; only 37 cases have been reported since
Some gastrointestinal anomalies associated with EA ! TEF 1874.94–100 Usui and colleagues101 reported a 47% incidence
are anorectal malformations (14%),64 duodenal atresia (2%),64 of tracheobronchial abnormalities such as ectopic or absent
intestinal malrotation (4%),74 ileal atresia, annular pancreas, right upper lobe bronchus, congenital bronchial stenosis, and
and pyloric stenosis. Genitourinary defects are varied and a decreased ratio of circumferential cartilaginous trachea to
include renal agenesis or hypoplasia (1%), hypospadias, membranous trachea; these abnormalities could play an impor-
undescended testes, cystic renal disease, hydronephrosis, vesi- tant role in such respiratory conditions as tracheomalacia
coureteral reflux, ureteric duplication, ureteropelvic and and atelectasis associated with EA.101 Several reports also sug-
vesicoureteral obstruction, urachal anomalies, ambiguous gest that EA is associated with abnormalities in vagal innerva-
genitalia, and cloacal or bladder exstrophy.59,75,76 Typical tion that are not related to surgical intervention to repair the
musculoskeletal malformations include limb (15%) and esophageal defect.102
vertebral (17%) anomalies.64
In 1973 Quan and Smith,77 suggested a broad spectrum
of associated malformations that are associated with EA Classification
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malformations. They arranged this association into the acro-
nym VATER: vertebral defects, anal atresia, tracheoesophageal As noted previously, numerous classification schemes have
fistula, esophageal atresia, and renal defects. Opitz defined been proposed and used to describe EA. The most useful
the term “association” as the idiopathic occurrence of multiple and practical classifications are perhaps simple anatomic
congenital anomalies during blastogenesis.47,77 Affected descriptions, even though many institutions and surgeons still
patients have no family history of malformations, no recogniz- use the Gross classification. It is important to note, however,
able teratogen is involved, and no chromosomal abnormality that there are many variations to the fairly straightforward
is observed. As the phenotype expanded, the acronym was anatomic descriptions given in Table 69-3. In 1976 Kluth35
changed to the VACTERL association, which includes verte- summarized and diagrammed many of the anatomic variants
bral, anorectal, cardiac, tracheoesophageal, renal, and limb not described by the many classifications mentioned here.
abnormalities.78 The incidence of the VACTERL association Waterston’s 1962 classification was based on risk factors
in the EA population is approximately 20%.65 Infants specif- (see Table 69-1). The classification affected the surgical care
ically with EA-TEF frequently have VACTERL anomalies of these patients. Infants in the “good”-risk category (A) were
(vertebral, 17%; anal, 12%; cardiac, 20%; renal, 16%; limb,
10%), as well as other midline defects (cleft lip and palate, TABLE 69-3
2%; sacral dysgenesis, 2%; urogenital anomalies, 5%).79 Incidence of Various Esophageal Atresia—Tracheoesophageal
The “renal” category should include urinary tract anomalies Fistula Anomalies
such as megalourethra, urethral duplication, urethral valves, 1. Esophageal atresia with a distal tracheoesophageal fistula 84%
stricture, and hypospadias. It generally requires a minimum 2. Esophageal atresia without tracheoesophageal fistula 6%
of three of the components to qualify as a “VACTERL patient.” 3. Tracheoesophageal fistula without esophageal atresia 4%
Recently, the VACTERL-H association has been described and 4. Esophageal atresia with a fistula to both pouches 1%
includes congenital hydrocephalus.77,80 Infants with EA mal- 5. Esophageal atresia with a proximal tracheoesophageal fistula 5%
formations in association with the VACTERL association have
898 PART VI THORAX
typically treated with immediate operative repair, “moderate”- adjunct in the prenatal diagnosis of EA anomalies suspected
risk infants (B) were managed by delayed repair, and “high”- on ultrasound.111
risk infants (C) were treated by staged repair. Although the Most infants with EA are symptomatic in the first few hours
Waterston classification continues to be used to compare results of life. The earliest clinical sign of EA is usually excessive
between centers, many investigators have questioned its current salivation that results from pooling of secretions in the phar-
validity in caring for these infants.68,92,103 With modern neona- ynx. Typically, the first feeding is followed by regurgitation,
tal critical care, more low-birth-weight infants are surviving and choking, and coughing. Other features are cyanosis with or
more treatment options are available for infants with multiple without feeding, respiratory distress, inability to swallow,
congenital anomalies. As a result, a search for modern criteria and inability to pass a feeding or suction catheter through
for survival has produced several new classification schemes. the mouth or nose into the stomach. If a distal fistula is
In 1989 Randolph and colleagues103 suggested a refine- present, the abdomen distends as inspired air passes through
ment in Waterston’s classification on the basis of the overall the fistula into the stomach. Pulmonary compromise can
physiologic status of an infant with EA. According to a report become significant as gastric fluid refluxes through the TEF,
analyzing the results of 39 infants managed with the physio- spills into the trachea and lungs, and subsequently leads
logic status criteria, more and earlier primary repairs were to chemical pneumonitis. As the abdomen distends with
performed, and excellent survival rates were maintained in air, the diaphragms (the principal muscles of respiration in
stable infants in comparison with 87 infants previously the newborn) elevate and pulmonary status worsens. Aspira-
managed by using Waterston’s criteria.103 tion of saliva from the upper pouch into the trachea further
Other prognostic classifications have recently been pro- exacerbates the pulmonary compromise.
posed. Poenaru and colleagues92 suggested that only severe The diagnosis of EA can be confirmed by passing a firm
pulmonary dysfunction with a preoperative mechanical venti- catheter through the mouth into the esophagus to the point
lation requirement and severe associated anomalies were inde- at which resistance is met. A few milliliters of air can be
pendent predictors of survival. Brown and Tam104 suggested injected through the tube and used as a contrast agent to dis-
that the measured length of the esophageal gap provides a tend the upper esophageal pouch as frontal and lateral films
method of classification to predict morbidity and long-term are obtained (Fig. 69-3). A flexible catheter such as a feeding
outcome. In a review of 357 cases of EA treated at the Hospital tube will often curl up in the upper pouch, also suggesting the
for Sick Children on Great Ormond Street in London between diagnosis of EA (Fig. 69-4). If necessary, 0.5 to 1.0 mL of
1980 and 1992, Spitz and colleagues93 found that birth diluted barium can be used as a contrast agent and injected
weight and major cardiac disease were important predictors into the upper pouch to confirm the diagnosis (Fig. 69-5).
of survival. The Spitz classification is currently the system most Under carefully controlled fluorography, barium may also
commonly used.65,105 Okamoto and colleagues196 recently detect a proximal TEF; in the usual circumstance of a portable
suggested a revision to the Spitz classification (Table 69-4). film performed in the neonatal unit, however, barium identi-
Using receiver-operating characteristic (ROC) curves to com- fied in the tracheobronchial tree is more likely to represent
pare the two classifications, these authors suggest that as overall contrast aspirated through the larynx rather than through a
care of low- and very-low-birth-weight infants improves, the proximal TEF. A very small or short upper blind pouch may
cardiac anomalies play an increasing role in assessing prognosis also suggest the presence of a proximal TEF. Air in the stomach
for infants with EA. and bowel confirms the presence of a distal TEF. Absence of air
in the abdomen typically represents isolated EA without distal
TEF (Fig. 69-6), but the presence or absence of a proximal
Diagnosis and Clinical Findings TEF must be confirmed by bronchoscopy. The incidence of
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proximal TEF is much higher than thought in the past. How-
Unlike many congenital pediatric surgical problems, prenatal ever, it is possible to have absence of intestinal air in the
EA is not commonly diagnosed. Prenatal detection of EA by circumstance of a narrow or occluded distal fistula, which is
ultrasonography relies on the finding of a small or absent identified at the time of operation.112,113 The diagnosis of
stomach bubble and associated maternal polyhydramnios.106 TEF without EA is more difficult and requires a high index
However, with these criteria, the predictive value of prenatal of suspicion on the basis of clinical symptoms. The diagnosis
ultrasonography in the diagnosis of EA is only 20% to is often delayed, and investigations are triggered by repeated
40%.107,108 The ultrasonographic finding of an esophageal coughing or choking during feedings, often with pulmonary
pouch in the middle of the fetal neck in association with poly- infiltrates on chest radiograph suggesting aspiration. The diag-
hydramnios and a small stomach may increase the accuracy of nosis can be made by barium esophagography in the prone
prenatal diagnosis of EA.109,110 A recent report suggests that position (Fig. 69-7); however, bronchoscopy with or without
real-time fetal magnetic resonance imaging may be a useful esophagoscopy is often required to confirm the diagnosis.
TABLE 69-4
Okamoto modification of the Spitz Classification: Predictors of Survival in Cases of Esophageal Atresia
Class Description Risk Survival
Class I No major cardiac anomaly, BW # 2000 g Low 100%
Class II No major cardiac anomaly, BW < 2000 g Moderate 81%
Class III Major cardiac anomaly, BW # 2000 g Relatively high 72%
Class IV Major cardiac anomaly, BW < 2000 g High 27%
CHAPTER 69 CONGENITAL ANOMALIES OF THE ESOPHAGUS 899
Preoperative Treatment
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Operative Repair
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A B
FIGURE 69-5 The upper esophageal pouch is outlined by 0.5 to 1 mL of thin barium in the frontal (A) and lateral (B) projections.
FIGURE 69-9 Extrapleural dissection is facilitated by using moistened tissue applicators or pledgets to push the parietal pleura away from the chest wall
and subsequently away from the posterior mediastinum.
902 PART VI THORAX
B C
D E
FIGURE 69-16 Thoracoscopic repair of esophageal atresia with a distal tracheoesophageal fistula. A, Position of the trocars, a 6-mm trocar for the
telescope and two 4-mm trocars for instruments. B, The posterior pleura has been incised and the upper pouch is being mobilized. C, The distal fistula
has been dissected free with the azygos vein left intact. D, Fistula being ligated close to the trachea. E, Completed end-to-end esophagoesophagostomy.
(From Bax KMA, Van der Zee DC: Feasibility of thoracoscopic repair of esophageal atresia with distal fistula. J Pediatr Surg 2002;37:192.)
CHAPTER 69 CONGENITAL ANOMALIES OF THE ESOPHAGUS 905
and small bowel has also been used in this situation. This ap- an electromagnetic field to pull together metallic “bullets”
proach, however, places the infant at risk for pulmonary col- placed in the two ends of the esophagus in order to shorten
lapse because each ventilator breath volume bypasses the lung the gap.
by preferentially passing through the TEF and escaping via the Other innovative operative techniques designed to narrow
low-resistance gastrostomy tube. The use of a gastrostomy the long gap before attempted surgical esophageal anastomo-
tube with an underwater seal may increase air resistance ses have been advocated. In 1971 Rehbein and Schweder139
and decrease runoff through the fistula. A small balloon cath- described the use of a nylon thread bridging the gap between
eter passed through the gastrostomy into the lower esophagus the two ends of the esophagus and attached to silver olives136
can provide excellent palliation for days to weeks.129a residing within each lumen. The olives were pushed toward
Emergent thoracotomy and fistula division in an infant each other over time until the two ends of the esophagus
with severe respiratory distress syndrome who requires pressed together and created a fistula. Shafer and David140
high-pressure ventilation is generally the best approach; in a used a similar technique to create a fistula between the two
premature baby, the decision to operate should be made early ends of the esophagus by simply approximating the two ends
because the hyaline membrane disease will worsen in the first of the mobilized esophagus with tacking sutures, then placing
24 to 36 hours and will make the lungs even less compliant. In a bridging silk suture. A modification of this technique has
many circumstances, the infant remarkably improves after recently been reported successful in five infants.141 Another
emergency fistula ligation; this improved condition may allow method that facilitates esophageal lengthening is the multi-
primary repair of the esophagus and thus eliminates the need staged, extrathoracic elongation technique of Kimura, in
for a second operation. which the upper part of the esophagus is mobilized and
initially brought out as an end cervical esophagostomy. Every
2 to 3 weeks, the esophagus and its cutaneous stoma are
LONG-GAP ESOPHAGEAL ATRESIA
surgically mobilized and translocated down the anterior chest
Occasionally in infants with EA-TEF, the upper pouch is high wall until enough length is achieved to perform an end-to-end
and the distance between the upper and lower esophageal esophageal anastomosis.142,143 Foker has described a novel
segments limits the ability to easily complete end-to-end eso- technique in which traction sutures on both the proximal
phagoesophagostomy with acceptable tension. The same is and distal esophageal pouches exit through the chest wall
true for most neonates with isolated EA because the lower and are serially pulled in opposite directions until the pouches
esophagus is not “attached” to the trachea. Although much approximate (Fig. 69-17). The external traction technique
has been said and written about the subject, there is no precise is reported to induce esophageal growth and expedite approx-
definition of “long-gap” EA. What is amenable to primary imation of the pouches, thus allowing for earlier primary
anastomosis by some surgeons may be considered “long repair (10 to 14 days).144,145 Internal traction sutures have
gap,” “very long gap,” or “ultralong gap” by others. In addition, also been used to promote esophageal growth in cases where
measurement of gap length can be biased by the method used the gap is such that an initial anastomosis would perhaps be
to measure the distance between the proximal and distal ends under too much tension. Successful thoracoscopic esophageal
of the esophagus. Some reports describe preoperative mea- elongation for long-gap EA has also been reported.146 There is
surement of the gap by inserting a radiopaque tube or bougie also a report of using the Kimura technique for proximal
into the upper pouch and placing contrast or a flexible endo- pouch lengthening and the Foker technique for distal pouch
scope into the distal pouch through a previously placed gas- lengthening.147
trostomy tube.130,131 Other definitions of gap length are based In addition to preoperative attempts to elongate the esoph-
on intraoperative measurements obtained before or after agus, many intraoperative techniques have been used to estab-
mobilization of the proximal pouch and, on occasion, the lish a primary esophageal anastomosis, either at an initial
proximal and distal pouches. These variations on the method operation in the newborn period or at the time of a delayed
of determining gap length contribute to the confusion and operation after the elongation methods previously described
debate about how long is long.132 have been used. Bagolan advocates using the “Foker traction”
In the case of isolated EA, in which a “long gap” is expected, principle intraoperatively, finding that significant length can
most pediatric surgeons would proceed with the operative be obtained after 20 to 30 minutes of traction on the esopha-
placement of a gastrostomy tube, a period of observation, geal ends.147a Livaditis and colleagues148,149 described the use
and an attempted delayed primary repair. It is well documen- of a circular myotomy of the upper part of the esophagus to
ted that during the first several months of life, the gap between decrease the tension on an end-to-end esophageal anastomosis
the two ends of the esophagus tends to lessen because of in a piglet model of EA and, in 1973, reported the clinical use
spontaneous growth, possibly related in part to reflux of bolus of this technique to lengthen the upper esophageal segment in
gastric feedings into the lower esophagus, which makes pri- order to allow for a primary anastomosis in an infant with
mary repair more feasible.133,134 In addition, many preopera- long-gap EA (see Fig. 69-11).150 Since that time, proximal
tive mechanical techniques have been described as a means esophageal circular myotomy to lengthen the upper esopha-
to facilitate narrowing of the esophageal gap. The most com- geal pouch and achieve a primary anastomosis in long-gap
monly used method is that of upper pouch bougienage, in EA has been widely used. The use of balloon catheters or
which a weighted bougie is passed through the mouth into cuffed endotracheal tubes inflated in the upper esophageal
the upper pouch, with forward pressure applied daily or twice pouch to facilitate mobilization and subsequent circular myot-
daily for 6 to 12 weeks before attempting delayed primary omy seems to be a useful modification of this method.151–154
repair.135 The use of preoperative upper and lower pouch We have occasionally made an additional right cervical inci-
bougienage to decrease gap length has also been described,136 sion to mobilize the upper esophageal pouch up and out of
but some of the early proponents have abandoned this tech- the neck and perform a more proximal second or third circular
nique.136a In 1975 Hendren and Hale137,138 reported using myotomy.132
906 PART VI THORAX
French needle
Roll 4–0 silk
(nasal pack) 4–0 silk
passing through
the thoracic
wall using
French needle One clip
Cotton roll
Application
of clips just
above the roll
Traction on
the suture
Two clips
A B
FIGURE 69-17 Foker technique for lengthening the upper and lower esophageal pouches by external traction sutures. A, External view of crossed
traction sutures and bolsters. B, Internal view. (From Foker JE, Linden BC, Boyle EM Jr, et al: Development of a true primary repair for the full spectrum
of esophageal atresia. Ann Surg 1997;226:533.)
However, significant complications of circular myotomy the technical details of this method to elongate the upper
have been described and have created a debate among pouch. Davenport and Bianchi163 reported good follow-up
surgeons regarding its safety. Complications include esophageal results in 25 infants with EA treated by this method over a
leak, impaction of food particles in the myotomized segment,155 5-year period. Variations of this technique have been des-
and mild ballooning of the myotomized segment. Severe bal- cribed and consist of the creation of a 5-mm posterior flap164
looning resulting in an esophageal pseudodiverticulum has or anterior esophageal flap,165 followed by an end-to-end
been reported to be a potentially serious complication. Several oblique anastomosis with a spatulated distal esophagus.
reports have noted the formation of a pseudodiverticulum with At the time of attempted initial or delayed primary repair,
a distal anastomotic stricture, as might be expected.152,155,156 the esophageal segments sometimes do not reach together
If the stricture is not treated, the pseudodiverticulum can even though one or more of the methods just presented have
enlarge so much that it causes complications such as dysphagia been tried. Several additional techniques have been described
and life-threatening respiratory obstruction.156 to facilitate an esophageal anastomosis. Despite the long-held
Several reports have suggested the use of a distal esopha- opinion that the blood supply to the distal end of the esoph-
geal pouch circular myotomy in addition to proximal myot- agus is tenuous and may be compromised by mobilization,
omy to achieve a primary esophageal anastomosis.157,158 many surgeons have found that the distal esophagus can be,
Many technical modifications have been suggested to mini- and often is, mobilized to facilitate a primary anastomosis
mize the risk for potential serious complications from circular (Fig. 69-18).166 We have successfully achieved primary
myotomy. The use of a spiral upper pouch myotomy with obli- end-to-end esophageal anastomosis by completely mobilizing
que suture closure of the muscular layer has been advocated as the distal esophagus down to and even through the esophageal
a technique that minimizes the chance of diverticulum forma- hiatus of the diaphragm. With this approach, some of the fun-
tion and, theoretically, improves motility.159,160 Another dus of the stomach can be brought up into the chest to facil-
method used to elongate the upper pouch involves the crea- itate anastomosis.132
tion of a full-thickness anterior flap of the upper pouch wall Taking this concept further, Schärli described a combined
as described by Gough; when folded distally, the flap can be abdominal and thoracic procedure in which distal esophageal
rolled into a tube and attached to the lower esophageal elongation was achieved by ligation and division of the left
segment.161 In 1989 Bar-Maor and colleagues162 emphasized gastric artery, transverse or diagonal division of the lesser
CHAPTER 69 CONGENITAL ANOMALIES OF THE ESOPHAGUS 907
A B
C D
FIGURE 69-19 A to D, Gastric transposition technique.
is instituted with or without daily upper pouch bougienage. traction-elongation technique can be undertaken. When the
Over an 8- to 12-week period, the process of esophageal elon- two ends of the esophagus are clearly too distant for primary
gation can be monitored by imaging studies that use contrast esophageal anastomosis despite preoperative attempts at elon-
material, metallic probes, bougies, or flexible endoscopy gation and consideration of these methods (i.e., lower esoph-
placed in the upper and lower esophageal segments agus does not reach above the diaphragm), we have proceeded
(Fig. 69-21, A and B). with a primary gastric transposition to the cervical esophagus,
When no further elongation progress is evident or when typically without a thoracotomy. In these latter circumstances,
the two ends of the esophagus can be brought to close prox- many surgeons prefer to proceed to a cervical esophagostomy,
imity, an operation is performed. Thoracotomy or a thoraco- preferably on the left side, with plans for a future esophageal
scopic approach with dissection and mobilization of the replacement procedure. Postoperative complications are sim-
proximal and distal esophageal segments is done and primary ilar to those in infants with repaired EA-TEF (see the later sec-
anastomosis is attempted. If these efforts do not allow primary tion on outcomes); these adverse effects primarily include
anastomosis, circular myotomy, hiatal mobilization, Kimura anastomotic leak and stricture, dysphagia, and gastroesopha-
procedure, proximal pouch flap esophagoplasty, or Foker’s geal reflux disease (GERD).
CHAPTER 69 CONGENITAL ANOMALIES OF THE ESOPHAGUS 909
A B
FIGURE 69-21 A chest film taken with a Replogle tube pushing in on the upper esophageal pouch and a contrast injection through the gastrostomy tube
demonstrates a long-gap isolated esophageal atresia (A). After 8 weeks, a repeat study with a bougie coiled in the upper esophageal pouch and a neonatal
flexible endoscope passed through the gastrostomy site and up the distal esophageal pouch allows for assessment of interval narrowing of the gap (B).
910 PART VI THORAX
nasogastric tubes. Division of the inferior thyroid artery and ESOPHAGEAL ATRESIA WITH AN UPPER
middle thyroid vein may be necessary to expose the plane POUCH FISTULA
between the trachea and esophagus. The recurrent laryngeal
nerve must be identified and preserved. Identification of the There are two versions of this anomaly: (1) proximal pouch
fistula is facilitated by encircling the esophagus with Silastic fistula in association with distal TEF (double fistula) and
slings, but it is important to be aware that the contralateral (2) proximal pouch fistula without distal TEF. Several reports
recurrent laryngeal nerve can be damaged during this have cited varying incidences of a fistula between the upper
maneuver. Once the fistula is identified (it is often higher esophageal pouch and the trachea. The exact incidence of this
than expected), traction stitches should be placed close to type of fistula has probably been underestimated because
the esophagus through the superior and inferior extent of some initially unrecognized proximal pouch fistulas have
the fistula to avoid rotation of the esophagus after division been reported as recurrent TEFs after repair of EA with distal
of the fistula. On the tracheal side, 5-0 polypropylene TEF,188 and some of these cases have been diagnosed as pure
sutures are placed at the superior and inferior limits of EA because the upper TEF was missed. A recent report by Bax
the fistula. The fistula is now divided close to the esophagus, and colleagues suggests that proximal pouch fistula without
and interrupted 5-0 Vicryl or PDS sutures are placed on the distal fistula is as high as 5.6% of all patients with EA.189
tracheal side to close the fistula. The esophageal side of the The diagnosis can be made by a preoperative proximal pouch
fistula is closed with absorbable polyglycolic acid sutures contrast study, preferably performed by a skilled pediatric
(Fig. 69-22). Some surgeons advocate interposing muscle radiologist under optimum conditions in the radiology suite
tissue between the two opposing suture lines to reduce using fluoroscopy and video recording or in the neonatal unit
the likelihood of recurrence of the fistula. A right thoracot- if necessary.190 This study usually shows a small upper pouch
omy or thoracoscopic approach can be used on the rare in addition to identifying the fistula. However, a frequent
occasion when the fistula is located well within the thorax point of confusion during this study and in the interpretation
or when a recurrent fistula from a previous EA repair is of results is the occurrence of spillover of contrast from the
being approached.187 pouch up into the larynx and down into the trachea. Because
Postoperative complications include respiratory distress of the potential for inaccurate interpretation, some surgeons
secondary to edema of the trachea or injury to the recur- rely on preoperative bronchoscopy to make or confirm the
rent laryngeal nerves. The degree of preexisting lung dis- diagnosis of a proximal pouch fistula (Fig. 69-23).190 Unfor-
ease and concerns about tracheal edema may warrant tunately, a small proximal fistula can also be missed when
leaving the endotracheal tube in place for several days after endoscopy is used. Another common approach to the diagno-
the operation. Esophageal leak and recurrence of isolated sis of this anomaly is complete mobilization of the upper
TEF are rare. pouch during repair of the EA in order to localize and repair
A B
FIGURE 69-22 H-type tracheoesophageal fistula. A, Anatomic relationship between the fistula and the recurrent laryngeal nerve. B, After division, the
fistula is closed by interrupted suture on both the trachea and esophagus.
CHAPTER 69 CONGENITAL ANOMALIES OF THE ESOPHAGUS 911
Complications
------------------------------------------------------------------------------------------------------------------------------------------------
Outcomes
------------------------------------------------------------------------------------------------------------------------------------------------
drainage can usually be achieved. This in turn allows sponta- operations (11% to 13%).123,206 Up to 13% of infants and
neous closure of the leak. Breakdown of the anastomosis is children will require removal of an esophageal foreign body
frequently followed by the formation of a stricture at the site after repair of EA with or without TEF, even in the absence
of the leak and is sometimes associated with a recurrent TEF. of a stricture on contrast studies.207 Parents have to be coun-
Major disruptions of the esophageal anastomosis account seled about slow introduction of solids and keeping the food
for only 3% to 5% of postoperative leaks and are typically minced until the child has enough molar teeth to grind the
recognized early (24 to 48 hours) after the initial repair. food adequately because the scar in the area of anastomosis
The infant frequently deteriorates from tension pneumothorax always remains.
or mediastinitis uncontrolled by drainage and antibiotics. Clinically significant narrowing at the site of the esophageal
Factors that probably contribute to anastomotic leak include anastomosis is traditionally treated by dilation performed via
poor surgical technique, ischemia of the esophageal ends, use antegrade or retrograde bougienage. We have found that pass-
of myotomy, and excessive tension at the anastomotic ing Savary-Gilliard type dilators prograde over a guidewire has
site.201,202 In this critical setting, reoperation for control of many advantages: This method allows fluoroscopic assess-
sepsis with adequate drainage and attempted repair of the ment during sequential dilations, enables the use of contrast
anastomotic leak are warranted. A pleural or pericardial patch, injection during the dilation session, and typically eliminates
with or without an intercostal muscle flap buttress, may help the need for rigid esophagoscopy. More recently endoscopic
secure anastomotic closure.203,204 If the esophageal anasto- balloon dilators have been frequently used with good
mosis is not repairable, cervical esophagostomy and delayed results.208,209 This technique has the theoretic advantage of
esophageal replacement may be required. producing a uniform and radial force at the site of the stricture
rather than the shearing axial force applied with traditional
Esophageal Stricture bougienage.210–213 Many strictures respond to one to three
Esophageal stricture is a common complication of anastomo- dilations (53%) in the first months after esophageal repair.
sis of the esophagus in EA, but the reported incidence varies However, a recalcitrant stricture resistant to repeated dilations
widely depending on the criteria used to define a stricture. will require resection and reanastomosis or even esophageal
In a recent large series, stricture requiring dilatation is replacement. Injection of triamcinolone at the stricture site
reported to occur in up to 80% of patients.68,105,116,147a Spitz during flexible esophagoscopy is used in many centers, mostly
and Hitchcock91 proposed that stricture be defined as the by pediatric gastroenterologists, but repeated injections may
presence of symptoms (dysphagia and recurrent respiratory lead to adrenal suppression. Recently, the application of
problems from aspiration or foreign body obstruction) and mitomycin C to the stricture under endoscopic control has
narrowing noted on endoscopy or contrast esophagography been reported to reduce stricture formation after dilation. It
(Fig. 69-25). Factors that have been implicated in the patho- is crucial to determine whether the esophageal stricture is
genesis of esophageal stricture include poor anastomotic tech- associated with GERD by investigation with contrast esopha-
nique (excessive tension, two-layered anastomosis, and silk gography, endoscopy, pH monitoring, or any combination of
suture material), long gap, ischemia at the ends of the esoph- these studies. Because all EA patients demonstrate GER to
agus, GERD, and anastomotic leak. Reports of thoracoscopic some degree, children are routinely prescribed histamine-2
repair of EA note stricture rates similar to many open blockers at the time of discharge. In the presence of an anas-
tomotic stricture or persistence of acid reflux, proton-pump
inhibitors may be preferable. Many strictures do not respond
to dilation attempts if severe GERD continues to bathe the
stricture with acid.212 When medical management of GER
fails and the stricture does not respond to esophageal dila-
tions, an antireflux procedure may be indicated. The stricture
is often successfully treated with dilations after the antireflux
operation; however, care must be given to not dilating the fun-
doplication because that significantly increases the possibility
of recurrent GERD. The most recalcitrant strictures have been
treated by resection and reanastomosis, or more recently by
stenting. However, experience is still limited with the latter,
and a fatal arterio-esophageal fistula from an unrecognized
aberrant right subclavian artery has been reported.*
Techniques that have minimized the likelihood of recurrent to be at risk for GERD and its complications,239,240 including
TEF include the use of a pleural flap,215 vascularized pericar- an apparent increased risk for the development of Barrett
dial flap,218–220 and azygos vein flap221 interposed between esophagus, perhaps as high as 9%.239–241 In a recent case con-
the esophageal and tracheal suture lines. Although recurrent trol study, 101 patients post EA repair with a mean age of 36
TEF typically occurs in the early postoperative period, it years (range 21 to 57) were compared with 287 controls. The
may not be recognized for months to years. Symptoms can authors found symptomatic GERD in 34% (vs. 8%), dysphagia
be typical of those seen with a congenital H-type TEF includ- in 85% (vs. 2%), and at endoscopy found an increased inci-
ing coughing and choking or cyanosis with feedings; however, dence of hiatal hernia (28%), Barrett esophagus (11%), esoph-
less obvious symptoms such as recurrent pulmonary infec- agitis (8%), stricture (8%), and epithelia metaplasia (21%);
tions are more common. The diagnosis may be suggested by manometry showed nonpropagating peristalsis in 80% and
an air-filled esophagus on plain radiographs of the chest. Rou- low distal wave amplitudes in all.242 One case of esophageal
tine contrast swallow studies will miss up to 50% of recurrent adenocarcinoma was reported in a 20-year-old patient in
fistulas. As with a congenital H-type fistula, contrast esopha- whom an EA had been repaired during infancy.243
gography performed in the prone position under videofluoro- The diagnosis of pathologic GERD is suspected in patients
scopy is a reliable method of establishing the diagnosis. with symptoms of vomiting, dysphagia, and recurrent anasto-
Bronchoscopy with cannulation of the fistula with a 2- to motic stenosis, which is occasionally associated with the
3-French catheter is also a reliable diagnostic approach and impaction of a foreign body or food bolus. In addition, respi-
is invaluable in locating the fistula during the operative pro- ratory symptoms such as stridor, cyanotic spells, recurrent
cedure. A recurrent TEF rarely closes spontaneously and pneumonia, and reactive airways disease may indicate GERD
typically requires surgical repair. Thoracotomy with fistula rather than other conditions such as tracheomalacia.
ligation and division is the operation of choice. To minimize The diagnosis of pathologic GERD in infants and children
the chance of recurrent fistulization, which has been reported after repair of EA is suggested by upper gastrointestinal con-
in 10% to 20% of patients with a first-time TEF recur- trast study. Twenty-four-hour pH probe data, though not as
rence, pleura, intercostal muscle, or pericardium should be standardized for children as they are for adults, typically
interposed between the esophagus and trachea.222 A recent document pathologic reflux.232 Extensive esophageal mano-
large series from the University of Michigan of 26 patients metric studies have consistently documented abnormal
over 27 years reports postoperative complications to include esophageal peristalsis and decreased lower esophageal sphinc-
leak (27%), esophageal stricture (15%), and recurrent ter pressures after EA repair; as a result, this test is probably
TEF (11%).223 not helpful in diagnosing GERD.244 Multichannel esophageal
Endoscopic eradication of TEF by means of various chemi- impedance combined with pH monitoring may emerge as a
cals or diathermy has been increasingly reported.224–226 superior test but is not widely available yet.245
Diathermy or laser deepithelialization followed by fibrin glue In infants and children with pathologic GERD, aggressive
has been reported in case reports and series with success rates medical management typically consists of thickening of
as high as 85%227,228; however, others continue to report fail- feedings, positioning of the infant in a prone or upright
ure with chemical techniques.229 A recent report of using posture, and administration of acid reduction agents such
argon plasma coagulation reports a 66% success rate.230 as histamine-2 blockers, proton pump inhibitors, and proki-
netic agents. However, 45% to 75% of these infants ultimately
undergo antireflux operations because of failed medical
LATE COMPLICATIONS management, failure to thrive, chronic pulmonary infection,
refractory anastomotic stricture, or the development of a distal
Gastroesophageal Reflux Disease
esophageal stricture.200,215,220 The choice of antireflux oper-
Gastroesophageal reflux disease (GERD) in infants and chil- ation is controversial. The Nissen fundoplication has typically
dren with repaired EA is common, and the incidence seems been considered the best option.234,246–248 However, debili-
to be increasing as suspicion and diagnostic investigation have tating dysphagia and significant complications including
become more common. In addition, the trend to preserve the wrap disruption and recurrent GERD in a third of patients
native esophagus with many of the techniques described ear- have been frequent after the 360-degree wrap.200,220,249,250
lier in this chapter also promotes GERD. The magnitude of the Because of the generally poor results with Nissen fundoplica-
problem is reflected in the finding that GERD occurs in 30% tion in this setting, some surgeons have used the anterior Thal
to 70% of patients after repair of EA.* The cause of GERD in partial-wrap fundoplication in these patients. However, as
this group of infants probably relates to shortening of the other surgeons have modified the Nissen fundoplication to
intra-abdominal portion of the esophagus because of anasto- create a short, floppy wrap (1 to 1.5 cm over a large dilator),
motic tension and/or esophageal motor dysfunction, either ac- this operation continues to be commonly used.246 In addition,
quired as a result of operative manipulation or intrinsic to the because the esophagus is frequently shortened in the setting of
congenital anomaly itself.232–234 The consequences of GERD repair of EA, some surgeons have used the Collis-Nissen
are then magnified by the poor or absent esophageal peristaltic fundoplication to gain intra-abdominal esophageal length.171
pump, thus exposing the esophagus to prolonged acid con-
tact. Esophagitis is frequently observed in older children Tracheomalacia
and adolescents on long-term follow-up of patients with Substantial respiratory symptoms occurring after repair of
EA.232,235–238 In several recent long-term follow-up studies, EA-TEF can be due to tracheomalacia. Tracheomalacia has
it appears that adults who had EA repaired as infants continue been noted to affect up to 75% of pathologic specimens from
patients suffering from EA-TEF; however, the condition is
*References 105, 147a, 200, 215, 220, 231, 232. problematic or significant in approximately 10% to 25% of
914 PART VI THORAX
with 30% having additional problems with choking.253,272,273 The second type of CES has been called idiopathic muscular
This disorder is clinically significant in that it is responsible for hypertrophy or fibromuscular stenosis and in some reports has
many long-term symptoms after EA repair including dyspha- been the most common form of CES.277 Its histologic charac-
gia, esophageal foreign body obstruction, GERD, and recur- teristics are submucosal proliferation of smooth muscle fibers
rent respiratory problems. Although these symptoms may and fibrous connective tissue with normal overlying squa-
improve with time, early problems with feeding intolerance mous epithelium.277 A resemblance to hypertrophic pyloric
and food bolus obstruction can lead to failure to thrive. Many stenosis has been suggested,277,283 but no clear embryologic
children must eat slowly and be selective with food choices. or pathogenic factors explain these lesions.
In a series of EA patients followed for 10 years, Lacher and CES caused by tracheobronchial remnants is described as
colleagues274 found 72% swallowed without problems; the most common type of CES in some reports and is certainly
however, 41% had poor weight gain (<25th percentile). the most described and understood of the three types of CES.
In addition, recent reports have examined long-term respi- It is believed that CES caused by tracheobronchial remnants
ratory outcomes after EA repair. One recent paper reports a occurs as part of a spectrum of anomalies, including EA-TEF,
greater than 60% incidence of recurrent respiratory infections, related to separation of the foregut from the respiratory tract
a 25% decrease in FEV1, and decreased maximal exercise per- around the twenty-fifth embryonic day.121 Tracheobronchial
formance at greater than 5 years beyond EA repair.275 Another tissue is believed to become sequestered in the wall of the
long-term follow-up study found respiratory symptoms in all esophagus and comes to reside in the typical distal location
patients including pneumonia 16%, bronchitis and asthma because of the faster growth rate of the esophagus.
52.3%, cyanosis episodes 9%, and cough 90%.194 In our CES secondary to tracheobronchial remnants was first
experience using small incisions and muscle sparing, thoracic reported in 1936 by Frey and Dusche284 in a 19-year-old
deformities are extremely rare. woman who died with the diagnosis of achalasia.121 In
1964 Holder and colleagues62 reported three cases of distal
Congenital Stenosis esophageal stenosis in their review of 1058 infants with EA,
TEF, or both. After several additional reports,285,286 Spitz,
of the Esophagus in 1973, first demonstrated a clear congenital basis for this
------------------------------------------------------------------------------------------------------------------------------------------------
disorder.287 In addition, 71 cases of CES have been reported
True congenital esophageal stenosis (CES) is a rare condition in the Japanese literature as of 1981279; 6 cases were reported
that has historically been confused with esophageal strictures in the Chinese literature (out of 76 total cases of CES in
secondary to inflammation, especially GERD. CES has been 1987).288 An additional three cases of CES have been
reported to occur once in every 25,000 to 50,000 births276,277; reported, one of each type described earlier.289
as of 2004, fewer than 600 cases have been described in the
world literature. For unknown reasons, the incidence of CES DIAGNOSIS
seems to be higher in Japan than in other parts of the
world.278,279 The incidence of other anomalies associated with Symptoms of CES usually begin in infancy with progressive
CES is reported to be 17% to 33%; these anomalies include EA dysphagia and vomiting, generally after the introduction of
with or without TEF; H-type TEF; cardiac anomalies; intestinal semisolid or solid foods around the age of 6 months. Some
atresia; midgut malrotation; anorectal malformations; hypospa- case reports, however, describe severe symptoms of regurgita-
dias; malformations of the head, face, and limbs; and chromo- tion and respiratory distress in the newborn.277 In some
somal anomalies.277 In addition, a report by Vasudevan and patients, a foreign body in the esophagus may be the first
colleagues280 noted that 5% of their patients with EA-TEF symptom noted.290 The correct diagnosis is frequently diffi-
anomalies had CES with tracheobronchial remnants, whereas cult to establish. Contrast esophagography typically reveals
other reports suggest an incidence as high as 12% to 14% of an abrupt distal esophageal narrowing, most often interpreted
patients with EA had associated CES.278,281 as a stricture related to GERD. Stenosis secondary to fibromus-
Classification schemes for CES have been numerous and con- cular hypertrophy can result in a more tapered narrowing
fusing, in part because of the difficulty in differentiating congen- (Fig. 69-27). CES caused by webs or fibromuscular hypertro-
ital from acquired lesions. The definition and classification phy can be manifested as midesophageal or even upper esoph-
proposed by Nihoul-Fekete and colleagues277 are perhaps the ageal stenosis.291 Over time, the esophagus proximal to the
most clear. CES is defined as an intrinsic stenosis of the esoph- stenosis can dilate, and contrast study results can be inter-
agus, present at birth, that is caused by congenital malformation preted as achalasia.292 Additional studies helpful in differen-
of esophageal wall architecture.277 This classification delineates tiating CES from achalasia and strictures caused by GERD
three forms of CES: (1) a membranous web or diaphragm, (2) include esophageal manometry and pH monitoring. Esopha-
fibromuscular thickening, and (3) stricture secondary to tra- goscopy typically shows esophageal narrowing with normal-
cheobronchial remnants in the wall of the esophagus. appearing mucosa at the level of the stenosis in cases of
A congenital membranous web or diaphragm is reported as CES. Recently, high-frequency endoscopic ultrasonography
the rarest of the three forms of CES.282 It has been considered has been reported to be helpful in the diagnosis of CES.293
to represent a missed form of EA25 and may be analogous to
membranes in other parts of the gastrointestinal tract. It is
TREATMENT
usually a partially obstructing lesion located in the middle
or lower portions of the esophagus. The membrane is covered Treatment of CES should relieve the symptoms of stenosis
on both sides with squamous epithelium and often has an and maintain the antireflux mechanism of the gastroesopha-
eccentric opening. Symptoms typically occur at several geal junction. Bougienage has been successful in treating
months of age as the infant begins consuming solid food. CES secondary to esophageal webs and fibromuscular
916 PART VI THORAX
RESULTS
Good long-term results have been reported for dilation and
operative resection.269 In patients who have undergone distal
esophageal CES resection without an antireflux procedure,
significant GERD has developed and required a subsequent
antireflux operation. Reported complications from treatment
by dilation include esophageal perforation and failure of ther-
apy. Complications of resection and anastomosis include
esophageal leak with mediastinitis, which can be successfully
treated by mediastinal drainage.270,272
To more adequately delineate therapy, several classification this method permits easy access to the cleft and allows asym-
schemes have been described. In reporting the first surgical metric incisions in the mucosa, thereby avoiding contiguous
repair in 1955, Pettersson described three types of clefts: suture lines. The major disadvantage to this approach is
type I, limited to the larynx and involving part or all of the its risk to the recurrent laryngeal nerves. The anterior laryn-
cricoid plate; type II, extending beyond the cricoid lamina geal approach exposes the larynx and upper part of the trachea
to the cervical trachea; and type III, involving the entire and has no risk for recurrent laryngeal nerve injury. Con-
trachea down to the carina. In 1991 Ryan and colleagues314 cern about postoperative laryngeal stability has been raised,
suggested a type IV in which the cleft extends beyond the and some surgeons recommend the use of stents during the
carina to involve one or both mainstem bronchi. healing process.310,316
Symptoms of LTEC vary depending on the extent of the Operative management for types III and IV LTEC requires a
cleft, but most patients immediately after birth exhibit respi- combined cervical and thoracic approach. Donahoe and col-
ratory distress aggravated by feeding. Additional symptoms leagues311,314,315 have described the use of a specifically
can include a characteristic toneless or hoarse cry, cyanosis, designed bifurcated endotracheal tube with flanged ends that
choking, increased secretions, and recurrent aspiration pneu- can be positioned during bronchoscopy to suspend the tra-
monia. It is not unusual for the severity of associated anoma- chea anteriorly with a ureteral catheter sling (Fig. 69-29).
lies to obscure the presence of LTEC, especially if it is a The endotracheal tube allows for confident airway control
minimal type I or II lesion. On the basis of the common symp- during the multiple position changes frequently required dur-
toms of LTEC, the diagnostic evaluation typically proceeds ing the operation. A right thoracotomy is performed, and the
along the line of the more commonly suspected diagnosis of tracheoesophageal cleft is exposed retropleurally. The tra-
EA-TEF or tracheomalacia. Contrast esophagography demon- cheoesophageal groove is incised on the right and opened
strates rapid confluence of contrast material in the upper part from the carina to the thoracic inlet. Separation of the two
of the esophagus and trachea; however, it is often difficult to tubes is completed by incising along the left side of the com-
know whether this is secondary to spillover at the level of the mon esophagotracheal wall, and an approximately 1-cm flap
larynx or passage of contrast through a cleft. Rigid endoscopy of esophagus running the length of the trachea should be left
under general anesthesia is the definitive method for diagnosis to help create the neomembranous portion of the trachea. It is
of LTEC, but it can still be difficult to identify a cleft unless the important to size the esophageal flap correctly in order to
index of suspicion is high and unless the frequent mucosal avoid stenosis or a floppy posterior wall, which can result
infolding at the level of the subglottic region is pushed open in tracheal obstruction. As the dissection proceeds, it is also
to reveal the cleft (Fig. 69-28). important to push away and thus protect the left vagus and
Management of infants with LTEC begins by maneuvers to recurrent laryngeal nerves. Closure of the trachea and esoph-
minimize aspiration and stabilize the airway. Ryan and col- agus is accomplished in a caudal-to-cranial fashion with run-
leagues314,315 recommended avoiding endotracheal intuba- ning polypropylene suture for the esophagus and interrupted
tion before surgery if possible, but it is often necessary to polypropylene for the trachea. A right cervical incision is then
intubate the trachea and perform tube gastrostomy before made to expose the upper portion of the esophagus, trachea,
definitive surgical repair of extensive LTECs. Operative proce- pharynx, and larynx; the repair is continued with inclusion of
dures vary depending on the severity of the cleft. Asymptom- a three-layer repair of the larynx and placement of a trache-
atic type I clefts may require no operative intervention, and ostomy tube, which can be custom-designed to avoid undue
symptomatic clefts have been successfully repaired endoscop- pressure against the posterior tracheal repair. Some surgeons
ically.310 Type II LTEC can be approached through a lateral prefer an anterior approach and divide the larynx and trachea
pharyngotomy, posterior pharyngotomy, or anterior laryngo- in the midline, as described for type II defects.317 Cardiopul-
fissure. The lateral exposure has been most often reported; monary bypass has been used both as a supporting technology
A B
FIGURE 69-28 Endoscopic view of a type III laryngotracheoesophageal cleft. A, Proximal end of cleft may be difficult to appreciate at first glance. B, Distal
end of cleft ends just proximal to the tracheal bifurcation.
918 PART VI THORAX
Sling in tracheostomy
site to catch tube
introduced from above
us Clavicle
ch
bron
L.
Pharynx
Esophagus opened
R
Extent of cleft
.b
ro
nc
hu
Tube secured anteriorly s
Holes cut
for upper B
lobe bronchi
A
C
FIGURE 69-29 Repair of a type III laryngotracheoesophageal cleft. A, Stabilization of a bifurcated endotracheal tube is done at bronchoscopy with a loop
passed through a tracheotomy, which draws the endotracheal tube forward. B, A cervical and thoracic approach allows retropleural exposure of the cleft.
A longitudinal incision is made in the right tracheoesophageal groove below the tracheal rings. The incision is extended inferiorly and across the esophagus
and up the left side, with approximately 1 cm of esophageal wall left attached to the trachea to allow adequate tissue to close the trachea. C, The trachea
has been closed with interrupted sutures, and the esophagus is closed in running fashion up to the thoracic inlet. Closure of the laryngeal portion of the
cleft and the lateral pharyngeal wall is not yet accomplished. (From Donahoe PK, Gee PE: Complete laryngotracheal cleft: Management and repair. J Pediatr
Surg 1984;19:143.)
during the anterior approach318 to repair a type IV cleft and as an inability to wean patients from the ventilator, pharyngoeso-
support in the form of extracorporeal membrane oxygenation phageal dysfunction, and GER are common postoperative
for one patient treated via a lateral thoracic and cervical complications. A reduction in morbidity and mortality de-
approach. pends, in part, on earlier recognition so that secondary com-
Few patients with these complex anomalies are treated at plications can be prevented. At present there is no clear
any single institution.310,315,319 Postoperative survival rates consensus about the best surgical approach; however, treat-
continue to be rather poor and range from 50% to 75%, ment is clearly complex and should be undertaken at centers
depending on the severity of the malformation, associated with multidisciplinary expertise.
anomalies, and prematurity. Anastomotic leakage is reported
to occur in approximately 50% of repairs and typically re- The complete reference list is available online at www.
quires reoperation via a different approach.310 In addition, expertconsult.com.