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 Review Articles - Artículos de Reseña
ABSTRACT
Esophageal atresia is the most com-
mon congenital anomaly of the esopha-
gus in newborns. This review article
discusses the incidence, embryologi-
cal classification, diagnosis and asso-
ciated anomalies of esophageal atre-
sia. Emphasis is placed in the current
guidelines of standard surgical mana-
gement of this congenital condition.
Index words: esophagus, atresia, gui-
delines, management
INTRODUCTION
Esophageal Atresia (EA) is the most common
congenital anomaly of the esophagus. Most cases
of EA are associated with tracheo-esophageal fistula
(TEF). More than three centuries has elapsed since the
first reported case. Following Leven, Ladd and Haight
adversities to accomplish successful repair of this de-
fect, the morbidity and mortality of EA has improved
in response to the development of neonatal intensive
care units (NICU), use of parenteral nutrition, better
surgical techniques, improved antibiotics and prenatal
diagnosis.1-4
INCIDENCE
EA occurs in one of every 2500 to 5000 live bir-
ths with a slight male predominance.5-7 Risk of having
a baby with EA increases with the first pregnancy, older
mother and twin pregnancy. Familial cases have been
reported.1, 6, 8, 9 Teratogenic influence has been identi-
fied after prolonged use of estrogen, progesterone or
thalidomide during pregnancy, and infants born to dia-
betic mothers. An experimental model of EA was de-
veloped in 1996 by exposing fetal rats to Adriamycin.
In 2004, the Adriamycin rat model was used to make
a correlation between EA, amniotic fluid volume varia-
tions and other visceral malformations.10
Chromosomal abnormalities are frequent in
children with EA. They include Trisomy 13, 18 (Edward)
and 21 (Down) along with the VACTERL association.7,
11, 12
A deletion of band 11.2 of the long arm of chromo-
some 22 has also been associated with EA.13 Recently,
a deletion in chromosome band 17q22-q23.2 was as-
sociated with esophageal atresia, tracheoesophageal
fistula and conductive hearing loss.14
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010
33
ESOPHAGEAL ATRESIA:
NEW GUIDELINES
IN MANAGEMENT
Jessica González-Hernández MS*
Humberto Lugo-Vicente MD**
From the * UPR School of Medicine, and ** Section of Pedia-
tric Surgery, Department of Surgery, UPR School of Medici-
ne, Puerto Rico Health Science Center, San Juan, PR.
Address reprints requests to: Humberto Lugo-Vicente MD
– PO Box 10426, San Juan, PR 00922. E-mail: titolugo@
coqui.net.
EMBRYOLOGY/CLASSIFICATION
The esophagus and trachea develop from the
foregut bud. During the fourth week of fetal develop-
ment the trachea forms as a ventral diverticulum of this
bud. Abnormal embryogenesis of the laryngeo-tracheo-
esophageal groove and subsequent septum formation
allows persistent communication between the trachea
and esophagus. The distal esophagus elongation oc-
curs with the heart and lung development reaching a
maximum length by the seventh week. The proximal-
third striated muscle develops from the pharyngeal
pouches and its blood supply comes from the thyrocer-
vical trunk. Aggressive surgical dissection of this por-
tion is permitted due to the submucous blood supply.
The middle and distal esophagus has smooth muscle
derived from dorsal mesenchymal tissue and its blood
supply arise from segmental roots of the aorta.15 Ag-
gressive surgical dissection of this segment leads to
ischemia.
Published evidence using the Adriamycin rat
model has revealed that EA forms as a blind-ending
pouch and the distal TEF develops as the middle branch
of a tracheal trifurcation. The distal foregut anlage is
switched toward a pulmonary phenotype, it trifurcates
and its middle branch grows caudally to fistulized into
the stomach.16 This theory encompassing a respiratory
origin to the distal esophagus explains the poor motility
observed, the presence of airway cartilage in the histo-
logy of the lesion and presence of ectopic esophageal
lungs.
Several variations of EA have been described
(see Figure 1). Most common variant anomaly (87%) is
proximal EA associated with distal TEF. The proximal
esophagus is hypertrophied and dilated from amniotic
liquid pressure. Impingement on the trachea by the
dilated pouch is the cause of faulty cartilaginous ring
development also known as tracheomalacia. Second
most common anomaly is pure EA (8%) associated
with a poorly developed distal esophagus. Following in
frequency is isolated TEF. The other variants are insig-
nificant and usually diagnosed intraoperatively.6
DIAGNOSIS
Prenatal diagnosis of EA using ultrasound relies
on the finding of a small or absent fetal stomach bubble
associated with maternal polyhydramnios. Using these
criteria’s, the positive predictive value is 56% and the
sensitivity of establishing the diagnosis 42%.17-18 Po-
lyhydramnios is most commonly seen in pure EA. Vi-
sualizations of the blind-ending esophagus during fetal
swallowing, known as the pouch sign, can also lead to
prenatal diagnosis.17, 19-21 Maternal alpha fetoprotein le-
vels has brought inconclusive evidence in the prenatal
diagnosis of EA.22
Most babies born with EA have symptoms since
the first hours of birth. They consist of excessive sali-
vation (mucosity), first feeding causing reflux, asphyxia
and cough followed by cyanosis, respiratory distress
and inability to pass an orogastric tube. The abdomen
could be either scaphoid or distended depending on
the presence of a distal TEF. The diagnosis of EA is
confirmed after watching a coiled orogastric tube in the
proximal esophagus in simple chest x-ray films. Con-
trast studies are rarely needed and of potential disaster
(aspiration). If in doubt a very small quantity (0.5 to 1
cc) of diluted barium can be instilled through the tube
to delineate the proximal esophageal pouch. A cons-
tant distance of 10-11 cm between the nares and proxi-
mal esophageal pouch is most often found. Abdominal
films should be obtained to rule out the occurrence of
associated gastrointestinal anomalies. Other diagnos-
tic modalities include esophagoscopy, fluoroscopy and
bronchoscopy. The presence of air in the gastrointesti-
nal tract suggests that a distal TEF is present, whereas
absent air makes the diagnosis of pure EA.1 Due to the
high incidence of associate malformations in children
born with EA, an initial evaluation should screen for
other defects using echocardiogram to exclude ductal-
dependent lesions, preoperative renal ultrasound if the
child has not voided and chromosomal analysis.
Congenital isolated TEF brings problems du-
ring early diagnosis and management. More than H-
type is N-type, due to the obliquity of the fistula from
the trachea (carina or main bronchi) to esophageal
side (see Figure 2) anatomically at the level of the neck
root (C7-T1). Pressure changes between both structu-
res can cause entrance of air into the esophagus, or
esophageal content into the trachea. Thus, the clinical
manifestation that we must be aware for early diagno-
ses are cyanosis, coughing and choking with feedings,
recurrent chest infections, persistent gastrointestinal
distension with air, and hypersalivation.17 Diagnosis is
confirmed with an esophagogram, or video-esopha-
gogram (high success rates, establish level of the
TEF). Barium in the trachea could be caused by as-
piration during the procedure. Upon radiologic doubt,
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010
34
bronchoscopy should be the next diagnostic step. De-
lay in surgery is generally due to delay in diagnosis
rather than delay in presentation.23
ASSOCIATED ANOMALIES
The pathogenetic mechanisms that produce
EA also affect other systems. Between 50 and 70%
of children born with EA have associated congenital
anomalies that affect prognosis and survival. Cardio-
vascular anomalies are the most commonly found
(29%), followed by genitourinary (14%), gastrointes-
tinal (13%), skeletal (10%) and chromosomal defects
(4%).24 Neurologic anomalies associated include neu-
ral tube defects, hydrocephaly, holoprosencephaly and
macrophthalmia. Other less common anomalies are
choanal atresia, cleft lip, abdominal wall defects and
diaphragmatic hernias. The association of two or more
system anomalies and the severity of associated ano-
malies influence mortality in esophageal atresia.3, 6, 24,
25
In 1973, the VACTERL association was descri-
bed. VACTERL is the most common association (14-
36%) seen in EA and includes:
• Vertebral anomalies
• Anal malformations
• Cardiac malformations
• Tracheo-Esophageal fistula (must be one of the as-
sociated conditions)
• Renal deformities
• Limb radial defects.
Other associations included within EA include
CHARGE (coloboma, heart disease, retarded develop-
ment/growth or central nervous system abnormalities,
genital hypoplasia or hypogonadism and ear abnorma-
lities or deafness), Downs, Potter, Fryns, Fanconi syn-
drome and others.3, 24, 26
Cardiac defects determine mortality. Risk of
death in an infant born with EA and a cardiac defect is
30%. The most common cardiac anomaly is ventricular
septal defect. Other cardiac defects include tetralogy
of Fallot, patent ductus arteriosus and anomalies of
the aortic arch.8, 27 In infants with non-duct-dependent
cardiac anomalies, repair of the esophagus takes pre-
cedence whereas in ductal-dependent lesions, tempo-
rary control can usually be achieved with prostaglan-
din E.6 Genitourinary anomalies include hypospadia,
undescended testis, renal agenesis, hydronephrosis,
vesico-ureteral reflux and ambiguous genitalia. The
most common gastrointestinal anomalies include im-
perforate anus, duodenal atresia, malrotation, annular
pancreas and pyloric stenosis.
Several deformities of the respiratory system
associated with EA can result in a fatal outcome. They
include an ectopic superior right bronchus associated
with a trifurcated trachea or tracheal bronchi, congenital
bronchial stenosis, tracheal, laryngeal and pulmonary
agenesis.23 Because these abnormalities may be re-
lated to the occurrence of respiratory disorders such
as tracheomalacia and atelectasis, early bronchosco-
pic examination may be useful in patient with predomi-
nantly respiratory symptoms.

Chromosomal anomalies such as trisomy 18

and 21 are rarely seen. The correction of EA in children
with Trisomy 18 brings challenging ethical questions
that should be discussed with the parents. Figure 1. Types and Frequency of Esophageal Malfor-
mations
SURGICAL MANAGEMENT
Preoperative Management

Repair of EA is not an emergency. Time should

be taken to optimize the physiologic state of the baby
before urgent repair. This includes secure the airway,
avoid further aspiration and manage the associated
pneumonitis (chest physiotherapy). An orogastric tube
(Replogle) set to low intermittent suction can remove
secretions from the upper esophageal pouch. The child
should be placed in a semi-sitting position to avoid gas-
tric content travel from the stomach to the lungs through
the TEF. Next, intravenous fluids and broad-spectrum
antibiotics should be administered. Lab work-up should
include hemogram, serum electrolytes, arterial blood
gases, and type and cross match for 20 cc/kg of weight
of packed red blood cells. Informed consent should be
obtained before surgery.

All patients with esophageal atresia should Figure 2. Isolated TEF

have an echocardiogram prior to surgery to assess the
position of the aortic arch. Operative repair should be
done through the contralateral side of the aortic arch.6

Premature babies with EA and respiratory dis-

tress from hyaline membrane disease in need of me-
chanical ventilation are a special group. Inefficient
ventilation due to preferential escape of air through the
TEF in the presence of stiff lungs exposes the child to
gastric perforation, abdominal compartment syndrome
and hypoxia. Temporary balloon occlusion of the dis-
tal esophageal segment has brought conflicting results
while emergent ligation of the TEF is the best manage-
ment alternative for the affected child (see Figure 3).6,
28

Intraoperative Management

As management for infants with esophageal

atresia has markedly improved, new surgical techni-
ques have been developed.29 In addition to the conven-
tional open repair, a minimally invasive thoracoscopic
repair was first successfully done by Lobe and Rothen-
berg in 2002.30-31 Many studies have been conducted
to establish the thoracoscopic repair in the new stan-
dard of care.32-34 Studies have concluded that thoracos-
copic repair is safe and comparable with conventional
open repair of esophageal atresia in terms of short-term
outcomes such as operative time, postoperative leak
and stricture rates.32 Long-term benefits of the thoracos-
copic repair needs to be further assessed since a pu-
blished study has failed to reveal the advantage in terms
of postoperative esophageal motor function.33
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010
35
Figure 3. Prematurity, Hyaline Membrane Disease and
Inefficient ventilation in a child with EA and TEF
Intraoperative management depends on type
of defect and associated malformations. The final ob-
jective is to establish bowel continuity while preserving
the native esophagus of the child.
The standard of care remains the open thora-
cotomy repair and at the operating room, the manage-
ment of EA with distal TEF entails the following guideli-
nes:
1. Thoracic approach should be contralateral to the
aortic arch, most often this means a right thora-
cotomy. A high axillary skin crease incision allows
unrestricted access to the posterior mediastinum,
carries minimal morbidity and provides excellent
scar cosmesis.35-36
2. Whenever possible preservation of the latissimus
dorsi and serratus anterior muscles should be done
(muscle-sparring thoracotomy) to avoid shoulder
deformity, scoliosis, and winged scapula.
3. Extrapleural approach to the posterior mediasti-
num is preferred since a rib does not have to be
removed. Anastomotic leaks using the extrapleural
approach are better tolerated.
4. Division of the azygous vein near the superior vena
cava entrance to help dissect the TEF is operator
dependant since many surgeons prefer not to liga- Figure 4. Surgical Repair of EA with TEF
te the azygous vein. The TEF is watertight ligated
with small absorbable sutures (6-0 PDS or Vicryl).
5. Anesthesiologist will help identify the proximal
esophageal stump while exerting pressure with
an oro-esophageal tube. The proximal esopha-
geal stump can be aggressively dissected up the
neck, but avoid using the cautery since recurrent
laryngeal nerve damage is a possibility. The distal
esophageal stump is dissected carefully since its
blood supply is more precarious.
6. A primary esophageal anastomosis between both
stumps can be accomplished most often using one
layer full thickness interrupted absorbable sutures.
Around eight stitches are needed. The surgeon
must develop judgement on the tension exerted
when performing the anastomosis. A small 5 Fr.
trans-anastomotic tube is placed in the stomach for
drainage and early gavage feeding. Once finished
a chest drain is left near the anastomosis and pla-
ced to underwater sealing suctioning (see Figure
4). Figure 5. Long gap EA, suction catheter in upper pouch
7. Intercostal closure and muscle apposition follows and gastrostomy
with transfer of the baby to the NICU.
than those who underwent an esophageal replace-
Management of the baby born with pure esopha- ment.37 Primary repairs in long gap esophageal atresia
geal atresia remains a challenge since it depends on would cause severe anastomotic tension and would
the distance (gap) between the esophageal stumps. increase the risk of leakage and stricture formation. A
There are different alternatives of treatment but an in- new approach has surged for these patients, “growth
dividually decision should be made for each patient. induction” of the esophageal stumps by internal or ex-
Initially, a gastrostomy is done. This will help start fee- ternal traction. A staged esophageal lengthening has
dings and permit measurement of the gap distance. been achieved requiring three operations but resulting
The proximal esophageal stump can be serially dilated in an esophagus-only repair with the gastroesophageal
using Bakes metal dilators, while bolus gastrostomy junction below the diaphragm.38 Further studies have
feeding will help the distal esophageal stump to grow demonstrated the efficacy of the growth procedure to
(see Figure 5). Meticulous nursing care is needed to treat the full spectrum of EA including those with most
avoid respiratory problems from chronic saliva aspira- rudimentary lower segments and the longest gaps. The
tion. Once a gap of one centimeter is obtained between long gap-EA patients treated by the growth procedure
the esophageal stumps, an anastomosis can be done. and an early active course of dilations and treatment of
GE reflux seem indistinguishable from the much more
Several techniques are described to gain leng- favorable short gap EA/TEF patients.39
th and accomplish a tension free anastomosis during
repair, such as circular or spiral myotomy, flap esopha- Isolated TEF management consists of surgi-
goplasty or extrathoracic extension, all performed on cal closure through a right cervical approach. A small
the proximal esophageal pouch.6, 8 Esophageal repla- guide-wire threaded through the fistula during bron-
cement can be accomplished using preferably colon, choscopy helps identified the fistulous tract. Working in
stomach or jejunum discussion of which is beyond the the tracheo-esophageal groove can cause injury to the
scope of this review. It has been proved that patient un- recurrent laryngeal nerve with subsequent vocal cord
dergoing primary repair had generally better outcomes paralysis. Recurrence after closure is rare.40
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010
36
Postoperative Care
With moderate or severe tension on the repair,
the child benefits from postoperative prophylactic pa-
ralysis and mechanical ventilation to avoid neck move-
ment, further tension, breakage or leak of the anasto-
mosis.6 The chest tube is left until it clogs or patency
of the esophagus is documented. The presence of
pneumothorax or saliva in the chest tube suggests an
anastomotic leak. Gavage feedings through the tran-
sanastomotic tube can be started after the third posto-
perative day. A barium swallow is performed seven to
ten days after the repair to document the presence of
leak, stricture, dysmotility and gastroesophageal reflux
(GER).
Since esophageal stricture is the one most fea-
red complication after esophageal repair, published
studies have tried to determine whether routine ba-
lloon dilatation of the anastomosis after repair of an
esophageal atresia with distal fistula is superior to wait
and see policy with dilatation only when symptoms ari-
se. Studies have reported that dilatation on demand
results in fewer procedures, fewer hospitals days and
likely lower cost.41-42
COMPLICATIONS
Repair of EA and TEF can be plagued with early
and late morbidity. The three most common anastomo-
tic complications are in order of frequency: stricture,
leakage and recurrent TEF. Late complications include
gastroesophageal reflux (GER), tracheomalacia and
dysmotility.43
Anastomotic strictures (or stenosis) are a
common finding after repair of EA. Incidence can be
as high as 50%. Predisposing factors include the use
of breaded silk, thepresence of GER and leakage of
the anastomosis. Strictures are associated with cho-
king, frequent respiratory infections and foreign body
impaction. Most children respond to dilatation. Tho-
se associated to GER should be fundoplicated.4, 17, 44
Esophageal leaks occur in 10-15% of all ca-
ses. Leaks that occur during the first 72 hours after
repair present as tension pneumothorax. An immediate
esophagogram should be done with water-soluble ma-
terials to exclude a complete esophageal breakdown
that might need repair, esophageal exclusion and/or
gastrostomy. Late leaks are usually small, present with
saliva in the chest drain and close spontaneously with
proper drainage and nutrition.17
Recurrent TEF after surgical repair for EA oc-
curs in approximately 3-15% of cases. Tension on the
anastomosis followed by leakage may lead to local in-
flammation with breakage of both suture lines enhan-
cing the chance of recurrent TEF. Once established, the
fistula allows saliva and food into the trachea, therefore
clinical suspicion of this diagnosis arises with recurrent
respiratory symptoms associated with feedings after
repair of EA. Diagnosis is confirmed with cineradio-
graphy of the esophagus or bronchoscopy. A second
thoracotomy is very hazardous, but is the most effecti-
ve method to close the recurrent TEF. Either a pleural
BOLETIN DE LA ASOCIACION MEDICA DE PUERTO RICO - VOLUMEN 102 NUMERO 1 - ENERO, FEBRERO Y MARZO DE 2010
37
or pericardial flap will effectively isolate the suture line.
A pericardial flap is easier to mobilize, provides suffi-
cient tissue to use and serves as template for ingrowth
of new mucosa should leakage occurs. Other alternati-
ves are endoscopic diathermy obliteration, endoscopic
chemocautherization, laser coagulation, or fibrin glue
deposition.40, 45-47
GER is commonly identified after successful re-
pair of EA. Constant gastric bathing of an esophageal
anastomosis causes inflammation, edema and strictu-
re formation. GER has been implicated in development
of esophageal leaks in the immediate postop period.
The presence of GER in EA is associated to shortening
of the esophagus and vagal nerve denervation during
repair. Stricture development, recurrent pneumonia,
failure to thrive, Barrett epithelium and severe esopha-
gitis are indications for fundoplication.4, 17, 48-51
Tracheomalacia refers to a structural/functio-
nal generalized or localized weakness of the tracheal
rings' support resulting in partial respiratory obstruction.
Most cases are associated with EA and as such flaccid
tracheal development after external pressure from the
dilated proximal blind esophageal segment has been
proposed as pathogenetic mechanism. Most cases
develop expiratory obstruction since only the intratho-
racic trachea if affected. The harsh barking cough is
the most characteristic initial symptom. Nutritional pro-
blems are the result of difficulty breathing as cyanotic
attacks might occur during feeding. Other incitatory
elements are intercurrent respiratory infections and as-
piration. Severe forms are characterized by life-threa-
tening apneic spells, inability to extubate the airways,
and episodic pneumonia. A cough and wheeze may
progress to complete airway obstruction and cyanosis.
Diagnosis is obtained with simple lateral thoracic films
(narrow slit-like appearance), bronchoscopy during
spontaneous breathing (antero-posterior narrowing in
expiration), cinetracheobronchography (allows extent
of tracheal collapse) or cine CT studies. Reflux must
be rule out and manage aggressively. For mild to mo-
derate symptoms no management is necessary as the
child will improve with time. For severe life threatening
tracheomalacia aortopexy must be undertaken. Failed
aortopexy may need tracheal reinforcement with auto-
logous cartilaginous grafts.6, 8, 52
RESULTS
Survival of EA has improved dramatically du-
ring the past fifty years. A general survival rate of 80 to
95% can be accomplished when children with severe
associated malformations are excluded. The original
Waterston’s risk classification has yielded to several
modifications. High-risk patients are those with very
low birth weight (less than 1500 grams), associated
cardiac anomalies, chromosomal defects, ventilator
dependant and long gap.
Although the long-term outcome of EA patients
seems favorable, respiratory and gastrointestinal symp-
toms plus functional abnormalities remain frequent. One
third of long-term patients report having impaired qua-
lity of life because of respiratory infections, dyspnea,
difficulties in swallowing and coughing at night.53 Al-
most 20% of patients have GER symptoms. The rate of
symptoms decreases with age. Gastric metaplasia and
esophagitis and the high rate of tracheal, esophageal,
and gastric inflammation suggest a need for long-term
follow-up. Gastric metaplasia is a premalignant lesion
of the esophagus.51, 54 Results can be classified as ex-
cellent if the child is totally asymptomatic, good if he
has occasional difficulty eating some foods. Children
with frequent deglutition or respiratory problems are
rated as regular results.
Growth and development after successful EA
repair remains within the 50th percentile. Children with
EA have more learning, emotional, and behavioral pro-
blems than children in the general population. A high-
risk group of children with major associated congenital
anomalies, who had been ventilated as a newborn,
were at special risk for cognitive problems.55 Children
with esophageal replacement procedures fare worst
that primarily repair cases. These children develop
anastomotic strictures, ulceration, bleeding, redundan-
cy and bezoar formation more commonly.
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RESUMEN
Atresia esofágica es la condición congénita más co-
mún del esófago en recién nacidos. Este articulo de
reseña discute la incidencia, clasificación embrioló-
gica, diagnostico y anomalías asociadas en atresia
del esófago. Se establece énfasis en los principios
actualizados del manejo quirúrgico de esta condi-
ción congénita.