Symposium on Pediatric Surgery I

Esophageal Surgery in Newborns, Infants and Children

Prema Menon and K.L.N. Rao
Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India

ABSTRACT
The most common surgery on the esophagus by pediatric surgeons the world over is performed in the newborn period in
babies with congenital esophageal atresia with tracheo-esophageal fistula. Post-operative complications like recurrent fistula,
anastomotic stricture and some patients with gastroesophageal reflux would also require surgical intervention. Apart from
esophageal dilatation, gastrostomy and feeding jejunostomy, children with strictures secondary to caustic ingestion, reflux or
previous esophageal anastomosis may require esophageal substitution. This operation may also be required in babies with
pure esophageal atresia as well as those with a long gap esophageal atresia with fistula. The entire stomach, stomach tubes,
colon or jejunum are often used but techniques preserving as much of the original esophagus as possible are preferable and
more physiological. Surgery is also required in children with congenital esophageal stenosis and duplication cyst. [Indian J
Pediatr 2008; 75 (5) : 939-943] E-mail : klnrao@hotmail.com
Key words : Esophageal surgery; Esophageal atresia; Esophageal replacement; Esophageal stricture; Thoracoscopy;
Esophagoplasty; Neonate; Child

Esophageal surgery is performed in all age groups in

children. The most common indication, esophageal atresia
(EA) is seen all over the world, especially so in our
country. In fact, surgery for EA is the most common
emergency surgery in neonates at our center, with about
180 cases per year. Healthy infants without pulmonary
complications or other major anomalies can undergo
primary repair in the first few days of life. Prompt
diagnosis with appropriate clinical management and
expeditious referral to a tertiary care center have led to
survival rates in this group of 100% percent.1 Due to the
sheer numbers, and inadequate intensive care facilities in
many parts of the country, overall results are not
comparable with western centers. Delayed presentations
are common but a lot of progress has been made over the
past few decades in units dedicated to neonatal surgery
even in this group.2 Long term follow-ups are available
even in babies who presented as late as on day 17 of life.3
While congenital anomalies form the bulk of the
indications for pediatric esophageal surgery, replacement
of the esophagus for acquired lesions like caustic strictures
are also performed, the techniques used being very
similar to those in adults.

forms an important part of the overall care.4 The baby

should be nursed with the chest in the upright position
and the oropharynx and upper pouch repeatedly
suctioned. Intravenous fluids, oxygen by hood and broadspectrum antibiotics are started. If there are features of
respiratory failure, the baby is intubated. Bag-mask
ventilation is not appropriate since it may cause acute
gastric distention requiring emergency gastrostomy.
Chest radiographs should be evaluated carefully for
skeletal abnormalities, cardiovascular malformations,
pneumonia, diaphragmatic hernia and a right aortic arch.
Before surgery, it is important to examine the babys
abdomen and perineum. An abdominal radiograph to
detect distal gas should be done as the surgical approach
would not involve a thoracotomy in babies with pure EA.
This is also evaluated for skeletal abnormalities, intestinal
obstruction and malrotation. A contrast upper
gastrointestinal series is not recommended. An
echocardiogram and renal ultrasonogram should be
obtained. The baby should be shifted to an intensive care
unit and operated after proper evaluation and
stabilization is achieved.

Correspondence and Reprint requests : Dr. K.L.N.Rao, Professor

and Head, Department of Pediatric Surgery, Advanced Pediatric
Center, P.G.I.M.E.R., Chandigarh, 160012, India. Ph: 0091-1722747585 Ext. 5320 (Off), 2715314 (Res), 09914208320 (M); Fax: 0091172-2744401, 2745078

The survival rate in babies with low birth weight,

pneumonia or other major anomalies is lower with cardiac
anomalies being the main cause of death. The Waterston
classification appears to be still relevant as a prognostic
indicator in our setup.5 These babies are managed with
parenteral nutrition, gastrostomy to prevent reflux of
gastric contents through the fistula into the trachea and
upper pouch suction until they are appropriate surgical
candidates.

Indian Journal of Pediatrics, Volume 75September, 2008

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Surgery in Neonate with Esophageal Atresia

Correct preoperative management of a baby with EA

Prema Menon and K.L.N. Rao

Bronchoscopy prior to starting of the procedure is
performed in many centers. A prospective study in our
department showed that it helped in identifying the
upper pouch fistula, distance of the lower pouch fistula
from the carina thereby helping placement of
endotracheal tube beyond the fistula during surgery as
well as identifying tracheomalacia. Although
postoperative pulmonary complications and ventilator
requirements were reduced, it did not alter the mortality
rate. 6
Surgical repair is performed under general anesthesia
with endotracheal intubation through a right
posterolateral thoracotomy. Frequent desaturation can
occur during surgery especially while retracting the lung.
An experienced anesthetist is therefore essential as these
babies should be manually ventilated with low
inspiration pressures and small tidal volumes. The azygos
vein is usually the first structure to be identified after
thoracotomy. As it lies over the tracheoesophageal fistula,
it is routinely ligated and divided. In a recent study, it has
been suggested that by preserving the azygos vein, early
postoperative edema of the esophageal anastomosis can
be prevented resulting in a significant reduction in the
number of anastomotic leaks.7
Post-operatively babies are nursed in a TEF chair, so
that elevation of the head end is always maintained at an
angle of 30-45o.8 We routinely start feeds on the 2nd postoperative day through a trans-anastomotic nasogastric
tube which has been inserted at the time of surgery.4
In long gap EA, babies often end up with an initial
gastrostomy and esophagostomy. Many complications
are encountered with a gastrostomy before the patient
gets an esophageal replacement. Although, it is routinely
advised to minimally dissect the lower pouch because of
its perceived deficiency of blood supply and to preserve
the vagal fibres, mobilization of the lower pouch can
bridge the defect in the first attempt and is far more
physiological than an esophageal replacement. In a
prospective, randomized study in our department, we
compared 20 neonates with long gap EA and TEF
managed by ligation of fistula, distal pouch mobilization
and primary repair versus ligation of fistula,
esophagostomy and gastrostomy followed by delayed
esophageal replacement. 9 The mean duration of
hospitalization as well as final survival was statistically
significant in favor of the first group (p<0.05).

difficulties occur. Recurrent tracheoesophageal fistula

would require surgical correction and is mostly seen at
the site of the primary anastomosis. Using a ventilating
bronchoscope, the fistula is cannulated with a ureteric
catheter which is then identified through
esophagoscopy. 10 Tissue damage of the poorly
vascularized distal esophagus and surgical dissection
performed too close to the trachea have been postulated
as risk factors.
Approximately one half of patients with surgically
corrected EA develop gastroesophageal reflux disease
(GERD). Of these, one half responds to routine medical
therapy with prokinetic agents, histamine H2 receptor
blockers, or both, while the other requires surgical
intervention in the form of fundoplication for correction.
Long-term endoscopic follow-up may be indicated in
these patients to look out for Barretts esophagus and its
sequelae. In a prospective study in our unit over a 2 year
period, 27 babies developed anastomotic leak. Creation of
a feeding jejunostomy in 71% of the patients allowed us to
maintain nutrition and also reduced the percentage of
feeds in intercostal chest drain on an average from 25% to
8 %, thus reducing chest contamination. There is a high
incidence of GER in these babies and an additional
gastrostomy gave better results than feeding jejunostomy
alone. (Monika Bawa, MCh thesis. Efficacy of
management protocol for anastomotic leak after repair of
EA and TEF, PGIMER. 2007).
Esophageal stricture
A contrast esophagogram is essential to know the
anatomy of the esophagus, presence of multiple strictures
as well as capacity and drainage of the stomach. In
patients with associated GER, this should be first
managed surgically before any definitive procedure on
the esophagus. Most respond well to further esophageal
dilatation. Localized strictures would require resection
and anastomosis. During dilatation, if an esophageal
opening cannot be found through endoscopy, a minilaparotomy and gastrostomy followed by retrograde
dilatation should be attempted before opting for organ
replacement / bypass procedures. Long strictures
especially secondary to caustic ingestion often require
esophageal replacement.
Esophageal Replacement

Most neonates who undergo repair of EA and TEF have

some degree of esophageal dysmotility which resolves
with time. Strictures at the site of the anastomosis are
common and may subsequently require dilatation. Serial
esophagography should be performed at two months, six
months and one year of age, or whenever swallowing

Esophageal replacement is indicated in children with pure

EA, long-gap EA when anastomosis is not possible,
corrosive strictures and other unusual causes. Type and
location of the graft depend on etiology and surgeon
preferences. Several options are now available with good
results, such as use of the native esophagus or
replacement with colon, stomach, or small bowel.
Common problems with all esophageal substitutions
include high stricture rate, leaks, GER and nutritional
problems. An optimal substitute for the esophagus should

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Indian Journal of Pediatrics, Volume 75September, 2008

Complications after esophageal atresia repair and their

management

Esophageal Surgery in Newborns, Infants and Children

preserve the native esophagus, have normal caliber, no
space occupying lesion in the mediastinum, preserve
gastric reservoir function, should withstand acidity and
have a shorter suture line. Spitz et al recently published
their results of 173 gastric transpositions through the
posterior mediastinal route.13 They observed that stomach
functions as a conduit rather than as a reservoir.
Nutritional problems as well as restricted pulmonary
function appear to be common features.
Native esophageal reconstruction is the procedure of
choice rather than esophageal replacement. A long-term
retrospective review of 21 out of 26 infants with pure EA
who underwent delayed primary anastomosis between
1977 and 2004 showed that this provides excellent
functional results in spite of the high incidence of GER
(66%) with 43% of these needing fundoplication.12 Sixteen
children developed strictures at the anastomotic site; 10
responding to repeated dilatations while 6 needed
resection and reanastomosis. At the end of the study
period, 15 out of the 17 survivors (88%) were on normal
diet with no respiratory problems while 2 (12%) were
dependent on gastrostomy feeds. There were four deaths
(19%) in this group. This method of management is based
on the natural growth of the esophagus with the interim
period managed by upper pouch suctioning and
nutritional support. The median age at operation was 80
days and the median hospital stay was 5.5 months which
may not be feasible in our setup.
Reconstructive methods using gastric tubes are
popular. Preparation of patient for gastric tube
esophagoplasty includes pre-operative contrast study via
gastrostomy or barium swallow, dietary advice i.e. high
protein diet with evidence of weight gain, as well as
assessment and management of co-existent cardiac and
pulmonary lesions. Sham feeding should always be
started after esophagostomy to develop oral feeding
mechanisms early and shorten time to complete oral
feeding after delayed esophageal repair. Counseling of
parents regarding 2 procedures, i.e. gastric tube
esophagoplasty followed by closure of esophageal ends
approx 6 weeks later, duration of hospital stay which is
likely to be more after the 2nd surgery, likely anesthetic
complications, chest complications, as well as possibility
of esophageal dilatations after completion of all stages
should be done well in advance.
We first reported our technique of fundal tube
esophagoplasty in 2003 in 4 patients where the entire
native lower esophagus is retained with the remaining
required length created out of the fundus as a tube in
continuity.13 This is not possible with any other substitute
except interposition grafts. The tube is brought out
through the neck in a retrosternal fashion. Postoperatively a chest X-ray should be performed
immediately after surgery (day 0). A few weeks later, a
contrast study is done through the neck stoma followed
Indian Journal of Pediatrics, Volume 75September, 2008

by esophago-esophageal anastomosis. Since native

esophagus is used, there is less chance of peptic ulceration
at the anastomotic site. More of the stomach is available
increasing intake and reducing chances of reflux. After
closure of the neck stomas, oral contrast study to see
caliber, stenosis, leak, stricture, reflux and stomach
capacity is performed. Clinically, patients should be
assessed for improvement in growth percentiles,
developmental milestones, respiratory tract assessment,
ability to take solids and liquids orally without difficulty
ad libitum. We have so far (2001-2007) performed this
procedure successfully in 28 patients having pure EA (9),
EA/ TEF with leak or long gap (16) and caustic strictures.3
Seven patients had prolonged salivary leak which was
managed conservatively with nutritional management
and esophageal dilatation with only one requiring
excision of fistula tract. Long-term complications have
included stricture in 3 cases requiring local resection in 2
and conversion to colon interposition in 1. Dysphagia (5)
mostly improved with time and 1-2 esophageal
dilatations. Reflux related problems were managed with
head up position, H2 blockers for 2 years at least and
fundoplication in all patients. Mobilization of esophagus
is sometimes difficult in patients with caustic strictures
and post leaking EA/TEF. Additional thoracotomy was
required in 2 children with caustic esophageal strictures.
Poorly placed gastrostomy tube with injury to
gastroepiploic arcade, odd location of cervical
esophagostomy, intraabdominal adhesions and small
capacity stomach create additional problems during
creation of the fundal tube.
Gastric tubes are simple to construct and long lengths
can be created. Neo angle of His can be created, and the
distal part of the tube remains in the high pressure
abdominal zone reducing chances of reflux.
Fundoplication can also be easily added to the procedure.
But anastomosis between cervical esophagus and antral
gastric mucosa increases likelihood of peptic ulceration at
anastomotic site. These reversed gastric tubes require
good stomach capacity so that sufficient length of tube to
reach the neck can be created, normal stomach drainage
and motility and availability of left gastroepiploic artery
and arcade. Advantages over colonic interposition include
rich blood supply, no tension on the interposed segment,
resistance of gastric mucosal tube to acid ulceration and
no bowel complications like diarrhea. Complications of
reversed gastric tube include cervical anastomotic leak,
stricture, narrowing at diaphragmatic hiatus, smaller
feeds due to reduced stomach capacity, acid reflux and
psychological difficulties. Our own long term results of
reversed gastric tube (n=10), show that although they
require multiple esophageal dilatations, and have
prolonged neck leak, ultimately almost all patients do
well and are able to take solids orally without difficulty.
In a series of 21 children who had isoperistaltic gastric
tube for varied indications, 16 could accept a normal diet,
941

Prema Menon and K.L.N. Rao

2 had significant dysphagia and 3 needed a feeding
jejunostomy. The esophagogastric anastomosis leaked in
2 cases, but both closed spontaneously. A temporary
dumping syndrome was encountered in two children.
Two patients had strictures of their upper anastomosis
responding to dilatations. The two patients who had a
pharyngogastric anastomosis developed either intractable
stricture or nonfunctioning anastomosis. One of them
died 9 months later from aspiration pneumonitis. Two
patients had cervical Barretts esophagus above the
anastomosis, indicating the need for lifelong endoscopic
follow-up. 14
Hadidi has developed a simple technique to improve
the results of colon replacement of the esophagus in
children. 15 In 5 neonates with long gap EA with or
without fistula and another 6 with long segment caustic
esophageal stricture, at the gastrostomy operation, the
segment of colon to be used for replacement was chosen
and the trunk of the corresponding vessel say middle
colic artery supplying the transverse colon was ligated
and divided proximal to the marginal artery. One to three
months later, this colon was used for esophageal
replacement. The follow-up ranged from 21-56 months.
By increasing the blood supply to the transverse colon
through the left upper colic and marginal vessels, the
success rate of colonic replacement improved and
minimized morbidity. Only 1 patient developed stricture
at the colo-esophageal anastomosis which required
resection and anastomosis six months later.
Thoracoscopy in EA and other esophageal surgery
The thoracoscopic approach to the treatment of EA is
being increasingly performed. Secondary effects like
thoracic cage deformities, winged scapula, or scoliosis are
reduced in comparison to the open technique. In a study
of 51 neonates with EA managed thoracoscopically, the
operative procedure took 90-390 minutes (mean 178
minutes) which is understandable. However, the stenosis
rate (45%) appears to be much higher than with the
conventional open technique. Postoperative leakage
occurred in 9 patients (18%). In this study, 6 patients also
underwent thoracoscopic aortopexy for tracheomalacia.
In 2 patients the thoracoscopic procedure had to be
converted to a thoracotomy.16
In a multi-institutional analysis of 104 newborns with
a mean weight of 2.6 kg (0.5), who underwent
thoracoscopic repair of EA, the mean operative time was
129.9 minutes 55.5 with 11.5% infants developing an
early leak or stricture at the anastomosis. 17 Thirty three
(31.7%) required esophageal dilatation at least once.
Conversion to an open thoracotomy was done in 5 cases.
However, 25 newborns (24.0%) later required a
laparoscopic fundoplication, the incidence of which
appears to be much higher than with the open technique.
Three patients died, one related to the EA/TEF on the
20th postoperative day. Although cosmetic results may be
942

better, no other major advantage has been demonstrated

by the laparoscopic route so far.
Compared to adults, achalasia is rare in children.
Laparoscopic Heller myotomy with a partial
fundoplication is presently the gold standard for
managing this condition in children.18
Other causes of esophageal obstruction
While evaluating a newborn with drooling of saliva and
respiratory distress, diagnosis other than EA should be
kept in mind. Traumatic instrumentation can produce
pharyngeal or upper esophageal perforation and an
attempt at passage of a catheter may produce a false
passage submucosally or a pseudodiverticulum.19 In the
absence of traumatic instrumentation a spontaneous
perforation of esophagus should be considered. Mostly
the mucosal tear heals spontaneously, however, the risk
of mediastinitis does exist. Rarely, thoracotomy may be
indicated. Esophagoscopy is contraindicated in these
cases as it may actually increase the size of the
perforation. Babies should be monitored for leukocytosis,
fever and swallowing abnormalities. Obstruction of the
lower end of esophagus can occur rarely due to congenital
esophageal stenosis which may respond to bouginage,
endoscopic excision of diaphragm or surgical resection
anastomosis. Enteric duplication cysts can occur in
relation to the wall of the esophagus rarely
communicating with its lumen. They can usually be
removed preserving the esophageal integrity by
thoracotomy or thoracoscopy. Esophageal intramural
pseudo-diverticulosis is another extremely rare entity in
children associated with hiatus hernia and benign
esophageal stricture secondary to GER. Patients present
with intermittent but slowly progressive dysphagia
especially for solids. They symptomatically respond to
esophageal dilatation and fundoplication.20
CONCLUSIONS
Esophageal surgery in neonates requires high degree of
surgical skills and good neonatal postoperative nursing
care. Post-operative problems are very common after any
form of esophageal replacement but success can be
achieved in almost all cases. However, the surgeon has to
be passionate towards the baby, tenacious and also give
moral support to parents who may get disillusioned with
the time taken for ultimate recovery. This is the most
important aspect for the surgeons who wish to care for
these children with esophageal substitutions.
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