Keywords
long gap - esophageal atresia - Foker - Kimura - multicenter
Introduction
For children at our two institutions with long-gap esophageal atresia (LGEA), defined
as a gap length of at least 5 cm, several techniques of esophageal lengthening were
used to achieve a primary anastomosis. Treatment of LGEA has proven difficult and
several approaches have been used including bouginage of both pouches with dilators
and time alone to achieve a delayed primary repair but, only a few studies have provided
data on efficacy and safety.[1]
[2] For those children in whom a spit fistula (SF) has already been created, the Kimura
advancement (KA) technique is an alternative and consists of a multistaged extrathoracic
elongation of the proximal esophagus by moving the cutaneous stoma progressively further
down the anterior chest wall.[3] More recently, Foker et al[4]
[5] described a technique (Foker technique [FT]) using traction sutures on the esophageal
segment(s) to induce growth until a primary repair was possible. To gain data about
these techniques and their combination, we pooled the experiences of two European
centers treating children with LGEA.
Materials and Methods
From 2007 to 2010, 15 children with LGEA were treated: 8 with pure EA, 6 with a lower
tracheoesophageal fistula (TEF), and 1 with an upper TEF. Nine children already had
a SF created elsewhere. At presentation, the gap ranged from 5 to 14 cm ([Table 1]). Patients were grouped according to whether or not a SF was present and by the
subsequent surgical strategies: Group A (no SF, n = 6) received FT on both pouches. Group B (with SF, n = 6) received KA of SF and FT of the lower pouch. Group C (with SF, n = 3) received closure of the SF and subsequent Foker technique (CSFT) on both pouches.
Table 1
Surgical data of all children treated with FT and KA
Group
|
1
|
2
|
3
|
4
|
5
|
6
|
7
|
8
|
9
|
10
|
11
|
12
|
A
|
HN
|
2
|
No SF
|
2
|
6
|
FT both pouches
|
2
|
None
|
Yes
|
2
|
None
|
None
|
|
LW
|
2
|
No SF
|
2
|
|
FT both pouches
|
2
|
None
|
Yes
|
2
|
None
|
None
|
|
JS
|
3A
|
No SF
|
2
|
|
FT both pouches
|
3
|
None
|
Yes
|
6
|
Stenosis
|
Complete rupture during second dilation, redo anastomosis
|
|
BS
|
2
|
No SF
|
5
|
|
FT both pouches
|
2
|
None
|
Yes
|
3
|
None
|
None
|
|
RC
|
3B
|
No SF
|
2
|
|
FT both pouches
|
2
|
None
|
Yes
|
1
|
Stenosis
|
Multiple dilations
|
|
LB
|
3B
|
No SF
|
5
|
|
FT both pouches
|
2
|
None
|
Yes
|
4
|
Stenosis
|
Stricture multiple dilations
|
|
Mean
|
|
|
3
|
6.5
|
|
|
|
|
3
|
|
|
B
|
WW1
|
3B
|
Right SF
|
10
|
|
KA, FT lower pouch
|
3
|
None
|
Yes
|
10
|
External leak (external fistula)
|
Necrosis of the lower pouch after hiatal hernia operation = jejunal replacement
|
|
OS
|
3B
|
Left SF
|
19
|
|
KA, FT lower pouch
|
4
|
None
|
Yes
|
12
|
Confined leakage, stenosis
|
Multiple dilations
|
|
WW2
|
2
|
Right SF
|
8
|
|
KA, FT both pouches (after conversion)
|
8
|
SF re-creation after severe infection
|
Yes
|
143
|
Large confined leak (no external fistula)
|
Stricture, perforation after dilation, stricture excision and redo anastomosis
|
|
AL
|
2
|
Right SF
|
26
|
|
KA, FT lower pouch
|
2
|
SF infection after Kimura procedure
|
Yes
|
2
|
External leak
|
Stricture excision and redo anastomosis
|
|
IW
|
3B
|
Left SF
|
30
|
|
KA, FT lower pouch
|
3
|
SF infection, thoracic empyema
|
Yes
|
48
|
Large confined leak (no external fistula)
|
Multiple dilations
|
|
AR
|
2
|
Right SF
|
5.5
|
|
KA, FT lower pouch
|
2
|
None
|
Yes
|
3
|
Stricture
|
Multiple dilations
|
|
Mean
|
|
|
16.4
|
|
|
|
|
|
36
|
|
|
C
|
TC
|
2
|
Left SF
|
8
|
|
CSFT closure, FT both pouches
|
4
|
Repetitive perforations of CSFT
|
Yes
|
18
|
Severe leakage
|
Redo 4 weeks after primary anastomosis
|
|
NL
|
2
|
Left SF
|
11
|
|
CSFT closure, FT both pouches, later conversion to KA
|
6
|
Severe mediastinitis, fluid overload (twice)
|
No
|
|
|
|
|
IM
|
3B
|
Right SF
|
13
|
|
CSFT closure, FT both pouches
|
5
|
Severe mediastinitis
|
No
|
|
|
|
|
Mean
|
|
|
10
|
|
|
|
|
|
|
|
|
Abbreviations: CSFT, closure of the SF and subsequent FT; FT, Foker technique; KA,
Kimura advancement; SF, spit fistula.
Notes: 1, identification; 2, type of EA according to Vogt; 3, the presence of SF; 4, age
at first elongation (months); 5, gap length (cm); 6, type of procedures: FT and KA;
7, number of thoracotomies during elongation; 8, complications during elongation;
9, primary repair; 10, traction time between first elongation and anastomosis (weeks);
11, early anastomotic complications; 12, late complications.
Results
In group A (mean age 3 months, mean gap length 6.5 cm), a primary anastomosis was
achieved for all six children after a mean traction time of 3 weeks and 2 to 3 thoracotomies.
In detail ([Table 1]), five patients required only one thoracotomy for placement of traction sutures
and a second for the esophageal repair. In one case (a 2-month-old boy with EA-type
Vogt 3a and a gap length of 7 cm), two thoracotomies were required: First, the upper
TEF was closed and the pouch was fixed internally to the vertebral column under slight
tension to allow healing. After 4 weeks, the internal traction was converted into
an external FT followed by primary anastomosis. In group A, there was only one late
complication; during the second dilation (5 weeks after the repair), the anastomosis
was severely disrupted, which required immediate excision of the stricture and reanastomosis.
This healed well without further complications. In group B, all six children (mean
age 16.4 months, mean gap length 9.5 cm) had a primary anastomosis, although it was
delayed in 2 (48, 143 weeks) because of infection. The elongation procedures required
a mean of 3.6 thoracotomies (range 2 to 8). In three patients, a SF infection occurred
after KA and in one child, an empyema from a lower pouch perforation during FT. Anastomotic
leakage occurred in five out of six cases and all healed spontaneously. Repeated dilations
were required in all and in two out of six cases, the anastomosis was redone because
of an intractable stricture. Finally, in one patient from group B, the distal esophagus
became necrotic after repair of a hiatal hernia and fundoplication which required
a later jejunal interposition.
In group C (mean age 10.6 months, mean gap length 6.5 cm) only one out of three patients
had a final anastomosis. In this group, major complications occurred during the elongation
procedures and the FT had to be aborted in two out of three. All three patients had
severe infectious complications following the closure of the SF from two leaks and
one iatrogenic perforation. Two cases (age 11 and 13 months with gaps of 7 and 5.5
cm) failed to achieve adequate length despite several attempts at reconfiguring the
traction sutures. One of these patients was converted from FT to KA of the upper pouch
without significant improvement. These two patients had a later esophageal replacement
(stomach, jejunum). Only one child age 8 months, with a gap of 7 cm) had an esophageal
anastomosis 18 weeks later. A significant anastomotic leak occurred, however, which
required five thoracotomies for drainage and ultimately a revision of the anastomosis.
Discussion
In children with LGEA, opinions differ significantly about the “best” solution for
the child.[1] Although there is general agreement that “one's own esophagus is best,” a strong
controversy exists how often this is possible and at what costs.[1] When a primary repair appears to be impossible or, at least, will be too long and
arduous a solution, some authors advocate an esophageal replacement by stomach, colon,
or jejunum.[1] Several approaches to achieve a primary repair have been used. In some cases of
LGEA, spontaneous growth of the segments will allow a delayed anastomosis with the
drawbacks being lengthy hospitalization and the risk of aspiration.[2] More recently, growth induction of the atretic esophageal segments using axial tension
provided by traction sutures has been used to achieve a primary repair and has provided
early follow-up data.[5]
[6]
[7] Even a combination of elongation techniques has been reported.[8] The data on the success rate of lengthening techniques, however, are limited and
to gain more insight into this problem, we pooled the experiences of two European
centers treating children with LGEA. This experience documents the use of growth techniques,
FT and KA, in solving the problem of providing esophageal continuity in LGEA.
Basically, our success rates depended on the gap length, the age of the patients,
and more importantly on the management of the SF. The best results were achieved in
infants (no newborns were treated) without a SF (group A) ([Table 1]). In this group, traction on both pouches resulted in an adequate length to perform
the anastomosis within a mean of 3 weeks, and these anastomoses showed the lowest
complication rate.
In group B, in which the FT and KA techniques were combined, an adequate length was
achieved for all children, but these repairs showed more complications despite being
operated on by the same surgeons with the same technique. Two patients had significant
infections from leaks during KA, which greatly delayed the anastomosis. These complications
raised a concern about whether or not a SF will affect the “quality” of the anastomosis
or its healing process. Possibly these esophageal segments contain more fibrous tissue
or if the blood vessels were not well preserved during the elongation. On the other
hand, the gap length was longer in group B than in group A and the children were older
at the time of the first FT (group A: mean of 3 months, group B: mean of 16.4 months).
Such comparisons raise the question of whether or not there is an optimal time period
for the FT or KA. Spontaneous growth of the esophageal segments may occur during the
first 3 months of life and traction-induced growth has been successful to over 2 years
of age but unfortunately, there are almost no experimental or scientific data about
this phenomenon.[2]
[5]
[7]
Problems also occurred with the SFs in group C. The length of the gap between groups
A and C did not differ (both 6.5 cm), but the age did (3 months vs. 10.6 months).
Group C experienced the most serious complications. Leakage from the closure site
and severe mediastinitis occurred in two patients during traction and in one from
an iatrogenic perforation. In two patients, the esophageal anastomoses could not be
performed. The leaks in the SF closes during traction (CSFT) make this approach more
risky and not generally advisable.
Another drawback of the FT is the number of thoracotomies and the accompanying surgical
trauma. Basically, the FT required only two thoracotomies, one to place the traction
sutures and other for the anastomosis.[5] Any revision of the traction sutures or surgical repair of complications, however,
will increase the number of thoracotomies. Our results showed that the mean number
of thoracotomies was 2 in group A and 3.6 in group B (range 2 to 8), due to complications
and traction sutures which pulled out. This is also no doubt that the FT is a more
demanding approach technically making the observed complications more likely and the
approach riskier initially.
What this study does not address is the long-term results especially in comparison
to the most common interposition grafts (stomach and colon as well as the much less
commonly used jejunum). Because the goal in pediatric surgery is 70 good years, the
answers to the questions surrounding the treatment of LGEA will require at least midterm
data 30 to 40 years from treatment. In the meantime, we recognize the potential problems
with FT and at one center (Leipzig) have formed an interdisciplinary team which together
with the parents discusses each case step by step to set clear goals and define criteria
for halting the procedure and going to an interposition graft.
From this experience we conclude: (1) Foker traction of both pouches (group A) produced
into a high rate of primary repairs in children with LGEA and no previous esophageal
operations. (2) The combination of KA and FT (group B) resulted in an equivalent rate
of primary repairs, but the number of thoracotomies and rate of complications increased
significantly. (3) CSFT (group C) resulted in a high complication rate. (4) More data
are needed, ideally from a multicenter registry, to elucidate the safety and efficacy
of each elongation technique and to establish an algorithm with clearer inclusion
and exclusion criteria.