Keywords
oesophageal atresia - tracheoesophageal fistula - thoracoscopy - neonate - outcome
Introduction
Oesophageal atresia (OA) with/without tracheoesophageal fistula (TOF) is a rare congenital
malformation occurring in 1 of 5000 newborns.[1] The survival rate of patients with OA has increased significantly during the last
decades,[2]
[3] and today exceeds 90%.[2] Most patients survive without associated severe malformations. The high mortality
of very low birthweight patients with TOF and patients with severe cardiac malformations
has also decreased significantly.[4]
In many surgical centers, open, right-sided muscle-sparing thoracotomy is the standard
approach for repair of OA/TOF.[3]
[5]
[6] The learning curve of the open approach is well known and the postoperative results
are predictable.[4]
[5]
Today, advances in pediatric thoracoscopy allow sophisticated procedures, even in
the confined anatomic spaces of neonates,[3] and thoracoscopic repair of OA/TOF has gained increasing acceptance. In 1999, the
first thoracoscopic repair of isolated OA was performed in a 2-month-old infant.[7] After 1 year, the first totally thoracoscopic repair of an atresia with distal fistula
was realized in a newborn.[8] These milestones allowed for a more widespread adoption of this technique and an
increasing number of pediatric surgical units are now performing minimally invasive
OA/TOF repair.[1]
[9]
Numerous authors reported on excellent feasibility of thoracoscopic OA/TOF repair.[1]
[3]
[6]
[9]
[10]
[11]
[12]
[13]
[14] However, the rate of postoperative anastomotic leak seems to be higher compared
with the open approach.[6]
[9]
[11]
In our center, thoracoscopic repair of uncomplicated OA/TOF is routinely performed
in carefully selected patients. The decision for thoracoscopy is based on precise
selection criteria. In case of any adverse events, the decision for a conversion is
made in close cooperation with the anesthetic team.
Aim of this study was to evaluate safety and efficacy of thoracoscopic repair of OA/TOF
using our selective approach. Endpoints were conversion rate and perioperative complications.
Patients and Methods
The study was approved by the Institutional Ethics Committee (approval number: 1306–2012),
and written informed consent was obtained from all guardians for anonymized data analysis
and publication. A retrospective chart review of all consecutive patients undergoing
OA/TOF repair between June 2001 and December 2011 was performed.
Data collected included birthweight, associated malformations, age at surgery, operative
time, conversion rate, and intra- and postoperative complications.
All patients underwent standardized preoperative assessment including full physical
examination, chest radiograph, echocardiogram, and renal ultrasound.
Criteria for the thoracoscopic approach were suspected short-gap OA/TOF, birthweight ≥ 2000 g, ≤ one
major associated malformation, and cardiorespiratory stability (no medical cardiac
support and no mechanical ventilation).
Operative Technique
Newborns were positioned semiprone with the right side slightly elevated and prepared
for open thoracotomy should this have proved necessary. Initially, a 5 mm port for
the videoscope was placed one intercostal space below the tip of the scapula, followed
by two 3.5 mm working ports placed under direct vision in the 7th to 8th intercostal
space paravertebrally in the anterior axillary line. Creating an intrathoracic pressure
of 3 to 5 mm Hg, CO2 insufflation was initiated with 1 to 2 L/min.
The azygos vein was not routinely dissected. Identification of the distal fistula
and suture ligation with 4/0 Vicryl was performed to facilitate ventilation. The upper
pouch was identified placing downward pressure on the nasogastric tube. Following
transection of the distal fistula, the first suture for the anastomosis was placed
and the knot tied to avoid tension. Hereafter, the proximal oesophagus was opened,
and a 6- to 8-Ch nasogastric tube was advanced through the distal oesophagus into
the stomach under direct vision. The anastomosis was performed circumferentially using
5/0 Vicryl interrupted sutures. A chest tube was not routinely inserted.
Standard perioperative monitoring included the following: pulse oximetry, electrocardiogram,
end tidal CO2 measurement, inhaled volatile agent concentration, invasive blood pressure measurement,
arterial blood gas measurement, and temperature.
In case of any intraoperative adverse events ([Table 1]) or lack of progress for ∼15 minutes, the procedure was converted to open muscle-sparing
thoracotomy.
Table 1
Reason for conversion from thoracoscopic approach for OA/TOF repair to open thoracotomy
Reason for conversion
|
No. of patients
|
%
|
Insufficient surgical exposition
|
2
|
9
|
Ventilation problems
|
3
|
14
|
Anastomosis under tension
|
2
|
9
|
Bleeding
|
1[a]
|
5
|
Total
|
8
|
36
|
Abbreviations: OA, oesophageal atresia; TOF, tracheoesophageal fistula.
a No need for blood transfusion.
Postoperative monitoring was performed in a pediatric intensive care unit. Weaning
and extubation was aimed at the earliest possible time point. Enteral feeding was
initiated on the first postoperative day. The nasogastric tube remained in situ until
day 10 post surgery. A contrast study was not routinely performed before initiating
oral feeds.
Data are quoted as median and interquartile ranges.
Results
A total of 44 consecutive patients with OA/TOF were treated in our center during the
study period. Total 22 patients (6 female, 16 male) met our criteria for thoracoscopic
repair of OA/TOF and underwent primary thoracoscopic approach.
A total of 22 infants (95%) had OA with TOF (n = 19 distal TOF, n = 1 proximal TOF, n = 1 both proximal and distal TOF) and one (5%) had isolated OA.
The mean birthweight was 2760 g (2020 to 3960). Seven of the patients (32%) were born
prematurely (≤ 36 weeks of gestation).
Concomitant anomalies were encountered in nine patients (41%) ([Table 2]). Age at surgery was ≤ 1 day (n = 2), ≤ 2 days (n = 6), ≤ 3 days (n = 11), ≤ 4 days (n = 2), and ≤ 5 days (n = 1). Mean operative time was 142 (75 to 220) minutes.
Table 2
Associated anomalies in the group of 22 patients undergoing thoracoscopic repair of
OA/TOF (anomalies were present in 9/22 patients)
Associated anomaly
|
No. of patients
|
Gastrointestinal malformations
Jejunal atresia
Imperforate anus
|
3
1
2
|
Cardiovascular malformations
ASD
VSD
|
7
4
3
|
Other
Trisomy 21
Tethered cord
Hydrocephalus internus
Vertebral anomalies
VUR > Grade 3
|
5
1
1
1
1
1
|
Abbreviations: ASD, atrial septal defect; VSD, ventricular septal defect; VUR, vesicoureteral
reflux.
Eight patients (36%) underwent conversion from a thoracoscopic procedure to an open
thoracotomy for various reasons ([Table 1]).
There was no anastomotic leak in the group of patients who underwent successful thoracoscopic
repair, but one recurrence of TOF ([Table 3]). This was the first patient of our series who underwent open revision and a successful
repair.
Table 3
Postoperative complications (thoracoscopic repair of OA/TOF versus converted operations)
in a group of 22 patients
Postoperative complications
|
Thoracoscopy
|
Conversion
|
Anastomotic leak
|
0
|
2 (9%)
|
Recurrent fistula
|
1 (5%)[b]
|
0
|
Anastomotic stricture[a]
|
4 (18%)
|
3 (14%)
|
Mortality
|
0
|
0
|
Total
|
5 (23%)
|
5 (23%)
|
a Requiring at least one dilatation.
b First patient of our series.
Anastomotic leak emerged in two patients (9%) in whom thoracoscopy had to be converted.
One leak could be managed conservatively and spontaneously resolved, the second patient
with a leak underwent thoracotomy and insertion of chest drain for pleural effusion.
An oesophageal stricture requiring at least one endoscopic dilatation emerged in 7
of the 22 patients ([Table 3]).
There was no mortality during a mean follow-up of 5.5 years (43 days to 10.6 years).
Discussion
Recent advances in minimally invasive surgery in infants and children have allowed
for a wide expansion of applications over the last decade,[1] hence thoracoscopic repair of OA /TOF has gained increasing acceptance.[6]
[10]
[15] The greatest advantage of a thoracoscopic approach is avoiding a posterolateral
thoracotomy in a neonate.[1] Our group and other authors have recently demonstrated that thoracoscopy in children
is associated with significantly less mid-term musculoskeletal sequelae additional
to a better cosmetic outcome compared with thoracotomy.[1]
[6]
[12]
[16] Furthermore, thoracoscopy provides an excellent visualization of the anatomy within
the confined spaces of the neonate because of its magnification which facilitates
the identification of the fistula.[1]
[6]
[12]
[13]
Despite all advantages, minimally invasive repair of OA/TOF remains a technically
challenging operation[6] with the suturing of the anastomosis being the major hurdle.[1]
[11]
It is a common consensus that surgical expertise and advanced thoracoscopic skills
are required to complete this operation successfully.[10]
[11]
[12]
Besides surgeon's expertise, we strongly believe that meticulous selection of patients
contributes to the success of this minimally invasive procedure. In the literature
available, no specific selection criteria have been established for the thoracoscopic
approach. We suggest the use of the aforementioned criteria to provide best possible
treatment of OA/TOF patients and to ensure a postoperative outcome at least similar
to the open operation.
It is well known that length of the oesophageal gap and associated major malformations
are important contributors to significant complications in OA/TOF.[5] A cornerstone of patient selection is a careful preoperative work-up. In our series,
the majority of the children (77%) were operated on day 2 or 3 of life after completion
of comprehensive diagnostic assessment.
It has been postulated that OA/TOF repair can be difficult in newborns ≤ 2000 g and
also in neonates with significant lung disease.[12] This is a part of the rationale of our concept using meticulous selection criteria.
The thoracoscopic approach was only performed in cardiorespiratory stable patients
with uncomplicated OA/TOF and a birthweight of ≥ 2000 g.
Comparable studies about thoracoscopic OA/TOF repair included patients with birthweights ≤ 2000 g
starting from 1000 g and severe congenital heart disease, such as double outlet right
ventricle and hypoplastic right heart.[6]
[9]
[11]
[12] Two newborns died after thoracoscopic repair of OA/TOF from severe congenital heart
disease, of whom one baby required an emergency intubation related to congenital heart
disease in which the endotracheal tube was thrust through the fistula closure on the
trachea after thoracoscopic repair.[12] Other authors reported on a 1.4 kg child that died postoperatively from further
undiagnosed anomalies.[9] In all cited studies, mortality is a relevant issue ([Table 4]). In our series, no deaths after thoracoscopic OA/TOF repair were observed, and
we believe that our concept of carefully selected patients contributes to this favorable
outcome.
Table 4
Comparison of our series with previous reports using thoracoscopy for repair of OA/TOF
Author
|
No. of patients
|
Conversion (%)
|
Anastomotic leak
|
Recurrent fistula
|
Anastomotic stricture[a]
|
Mortality (%)
|
van der Zee and Bax[6]
|
51
|
4
|
18%
|
4%
|
45%
|
2
|
MacKinlay[9]
|
26
|
4
|
27%
|
4%
|
35%
|
12
|
Patkowsk et al[11]
|
23
|
0
|
13%
|
0%
|
17%
|
13
|
Holcomb et al[12]
|
104
|
5
|
8%
|
2%
|
32%
|
3
|
Szavay et al[14]
|
25
|
32
|
4%
|
4%
|
n.a.
|
n.a.
|
Current study
|
22
|
36
|
0% (9%)[b]
|
5%[c]
|
18% (14%)[b]
|
0
|
Abbreviation: na, not available.
a Requiring at least one oesophageal dilatation.
b In cases where thoracoscopy had to be converted to open thoracotomy.
c First patient of our series.
Furthermore, we believe that our concept of early conversion to thoracotomy in case
of any adverse events contributes to a low rate of complications. Compared with other
studies ([Table 4]), conversion rate in our series was high.[6]
[9]
[11]
[12] Only one other study by Szavay et al[14] reports a similarly high conversion rate. The main reason for conversion mentioned
by the authors was increasing intraoperative hypoxemia, following the concept of a
selective approach and consideration of other than surgical aspects.
Anastomotic leak can be devastating and may result in mortality as a result of mediastinitis
and irreversible sepsis.[5] Tension on the suture line is obviously a contributing factor to the possibility
of a leak.[5] In our study, an anastomosis under tension was one of the reasons for conversion.
The incidence of anastomotic leak in our series was low and compares favorably with
other reports in literature on conventional operation,[6]
[9]
[11] ([Table 4]). The two observed leaks only occurred after conversion reflecting difficult intraoperative
circumstances.
Anastomotic stricture represents the most common cause of revisional surgery in children
with OA/TOF.[5] The majority of the studies report a stricture rate between 17 and 45%,[6]
[11] ([Table 4]). In our series, the rate of an anastomotic stricture requiring at least one dilatation
is comparable to the reported numbers. However, all strictures responded well to endoscopic
dilatation and none of the patients needed operative revision.
Conclusion
Thoracoscopic repair of OA/TOF remains a technically challenging operation. However,
using standardized meticulous patient selection and prompt conversion in case of any
adverse events, thoracoscopic repair of OA/TOF can be safely performed achieving excellent
results and a low rate of complications.