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Infants with repaired esophageal atresia and distaltracheoesophageal fistula with severe respiratory distress:is it tracheomalacia, reflux, or both?

Ahmed Nasr, Sigmund H. Ein, J. Ted Gerstle*

Division of General Surgery, Hospital for Sick Children, Toronto, Ontario, Canada M5G 1X8

0022-3468/$ – see front matter D 2005

doi:10.1016/j.jpedsurg.2005.03.001

Presented at the 56th Annual Meetin

American Academy of Pediatrics, Sa

8-10, 2004.

T Corresponding author. Tel.: +1 416

E-mail address: ted.gerstle@sickkid

Index words:Esophageal atresia;

Tracheomalacia;

Gastroesophageal reflux

AbstractAim of Study: Infants with repaired esophageal atresia and distal tracheoesophageal fistula (EA/TEF)

are at risk for severe respiratory distress, which is related to tracheomalacia (TM), gastroesophageal

reflux, or both. This usually mandates an operation for TM and/or a fundoplication procedure (FP).

Methods: We retrospectively performed a 26-year review of 288 patients with repaired EA/TEF.

Research Ethic Board approval was obtained.

Results: Postoperatively, 22 (7.6%) infants with EA/TEF developed severe respiratory distress. Thirteen

infants had an initial TM procedure and symptoms improved in 7 (54%). The 6 (46%) remaining

patients with ongoing respiratory symptoms required an FP. Nine infants had an initial FP and the

symptoms improved in 6 (67%). The remaining 3 (33%) patients with ongoing respiratory symptoms

required a TM procedure. All patients improved with the second procedure. Several clinical parameters

were assessed among the 4 patient groups (FP only, FP followed by TM procedure, TM procedure

only, and TM procedure followed by FP); there were no significant differences noted.

Conclusions: Whereas 54% to 67% of infants improved with a TM procedure or FP, 33% to 46%

required both surgical procedures. No clinical parameters were identified that could predict which

procedure should be performed first.

D 2005 Elsevier Inc. All rights reserved.

Infants with repaired esophageal atresia and distal

tracheoesophageal fistula (EA/TEF) are at risk for severe

respiratory distress (pneumonias, bblue spells,Q and respi-

ratory arrests). These events are related to tracheomalacia

Elsevier Inc. All rights reserved.

g of the Section on Surgery of the

n Francisco, California, October

813 6401; fax: +1 416 813 7477.

s.ca (J.T. Gerstle).

(TM), gastroesophageal reflux (GER), or both and mandate

a procedure for TM, aortopexy or tracheal stent placement,

and/or a fundoplication procedure (FP). In the literature,

there are several published works that have suggested TM

as the primary cause of respiratory distress in infants after

EA/TEF repair; fewer authors have implicated GER as the

primary factor behind these life-threatening complications

[1,2]. No studies have examined the role of surgery to

address these respiratory complications when both TM and

GER are present simultaneously. We reviewed our experi-

Journal of Pediatric Surgery (2005) 40, 901–903

Table 1 Patients treated with a procedure for TM (aortopexy or tracheal stent)

Age at

presentation (d)

Sex Presentation of

respiratory distress

Associated congenital

anomalies

Age at

procedure (d)

Procedure

for TM

162 F Blue spell None 164 Tracheal stent

1428 F Blue spell Cardiac 1440 Tracheal stent

27 M Respiratory arrest None 30 Aortopexy

111 M Blue spells Anorectal malformation 120 Aortopexy

75 M Blue spells None 90 Aortopexy

35 M Respiratory arrest None 37 Aortopexy

130 M Respiratory arrest None 150 Aortopexy

A. Nasr et al.902

ence to determine the optimal surgical management of

those children with EA/TEF who have both complications.

1. Methods

We retrospectively reviewed the charts of 288 infants from

1977 to 2003 who had EA/TEF repaired at The Hospital

for Sick Children in Toronto, Ontario, Canada. Postopera-

tively, 29 (10 %) patients developed severe respiratory dis-

tress, defined as the development of pneumonias, cyanotic

episodes, blue spells, or respiratory arrests in the absence of

underlying cardiac disease. Radiological investigations and

endoscopic procedures confirmed the presence of significant

TM and GER in 22 cases, whereas in 7 cases, they confirmed

either significant TM or GER. In the 22 cases where both

diseases coexisted, it was not possible to determine which

was the major cause of the observed respiratory distress.

2. Results

After repair of the EA/TEF, 22 (7.6%) infants developed

severe respiratory distress, which was related to both TM

and GER, at 204 F 330 days of life. All findings were

confirmed by radiological investigations (upper gastrointes-

tinal swallow) and endoscopic procedures (upper endoscopy

and/or bronchoscopy). All other causes such as cardiac

anomalies and recurrent TEF were excluded as causes of

these respiratory symptoms. The decision to proceed with a

Table 2 Patients treated with fundoplication

Age at

presentation

(d)

Sex Presentation

of respiratory

distress

Associated

congenital

anomalies

Age

at procedure

(d)

62 F Blue spell None 65

900 M Blue spell Anorectal

malformation

990

360 M Blue spell Cardiac 630

119 F Pneumonia Anorectal

malformation

120

25 F Respiratory

arrest

None 30

201 M Respiratory

arrest

Cardiac 210

specific surgical procedure was based on personal clinical

impressions that were unique to each surgeon. Thirteen

(59%) of 22 infants had an initial TM procedure and the

respiratory symptoms improved in 7 (Table 1). Six (46%) of

13 patients with ongoing respiratory symptoms required an

FP within 60 F 36 days of the TM procedure. All of the

patients improved with the second procedure (Table 3).

Nine (41%) of 22 infants had an initial FP and the

respiratory symptoms improved in 6 (Table 2). Three (33%)

of 9 patients with ongoing respiratory symptoms required a

TM procedure within 160 F 176 days after the FP. All of

the patients improved with the second procedure (Table 3).

Several parameters were assessed among 4 patient

groups (TM procedure only, TM procedure followed by

FP, FP only, and FP followed by TM procedure): clinical

presentation, associated anomalies, and length of time to the

first and second procedures. There were no significant

differences between the groups for any of these parameters.

3. Discussion

We still believe in the old teaching that the original

esophagus, albeit not properly formed, is infinitely better

(after its repair) than any substitute. We further realize that

EA surgery will have its share of complications. Anasto-

motic leak is the most worrisome complication, but others

include TM, anastomotic stricture, foreign body obstruction,

recurrent TEF, missed TEF, dysmotility, and GER [3]. A

small number (10%) of these infants some weeks or months

after repair will have severe sudden respiratory symptoms

(pneumonia, blue spells, or respiratory arrests), some of

which are felt to be initiated by feedings [2]. The occasional

bcrib deathQ has occurred in this group of babies with EA/

TEF with an incidence somewhat greater than by chance [3].

The cause of these clinical symptoms is said to be related to

an obstructed or flattened trachea between the innominate

artery and the aortic arch anteriorly and the distended upper

esophageal pouch (during feeding) posteriorly. Whether this

is actually mechanical in origin or indeed reflex apnea (a

term coined by Fearon and Shortreed [4]) or due to GER

into the oropharynx and into the trachea is a good question

that is yet to be answered.

Both TM and GER are very common in children who

have undergone surgery for EA/TEF [5]. When both

Table 3 Patients who required both a fundoplication and a procedure for TM

Age at

presentation (d)

Sex Presentation Associated congenital

anomalies

Age at 1st

procedure (d)

Age at 2nd

procedure (d)

TM procedure Procedures

68 F Respiratory

arrest

None 72 111 Aortopexy TM/FP

55 F Blue spell Anorectal malformation 56 96 Tracheal stent TM/FP

118 M Pneumonia Duodenal web 120 160 Tracheal stent TM/FP

88 F Respiratory

arrest

Cardiac 90 210 Tracheal stent TM/FP

199 M Blue spell None 210 240 Aortopexy TM/FP

55 M Respiratory

arrest

None 60 150 Aortopexy TM/FP

27 F Respiratory

arrest

Anorectal malformation 30 120 Aortopexy FP/TM

60 M Respiratory

arrest

Cardiac 61 91 Aortopexy FP/TM

77 F Blue spell None 90 450 Aortopexy FP/TM

TM/FP indicates TM procedure followed by FP procedure; FP/TM, FP procedure followed by TM procedure.

Infants with repaired EA/TEF with severe respiratory distress 903

conditions coexist, it is difficult to determine which is

responsible for the severe respiratory symptoms; this was

true in the cohort of patients examined in this study. The

choice of the first operation (TM procedure or FP) was each

surgeon’s personal preference according to what was felt to

be the underlying cause.

The incidence of severe TM in patients with EA/TEF has

been reported as high as 16% by Spitz et al [5] and 33% by

Slany et al [6]. Filler et al [1] in his 1992 publication stated

that a TM procedure (either aortopexy or tracheal stenting)

is the procedure of choice for infants presenting with severe

respiratory problems. We agree with this observation once

significant GER can be safely excluded.

It has been stated that about 80% of patients with EA/

TEF will have GER, but only 20% of them will be

symptomatic, requiring treatment. In such symptomatic

patients who fail maximal medical management, surgical

management with an FP is necessary [7,8]. The primary

drawback to an FP in these patients is related to the poor

peristalsis of the distal esophagus; the FP can make the

distal esophagus relatively obstructed, worsening the

patient’s clinical condition [9,10]. Infants presenting with

severe respiratory distress (pneumonia, blue spells, or res-

piratory arrests) after previous repair of EA/TEF can be

a diagnostic dilemma, if both TM and GER are present.

In such situations, the surgeon must choose between 1 of

2 types of surgical procedures based on no solid clinical

evidence. It is important to exclude other causes of severe

respiratory distress such as cardiac abnormalities and

recurrent TEF. It is presumed that for the infants who

have clinically improved with a procedure for correction

of TM, the continuing severe GER will be better handled

by a noncollapsing trachea. Similarly, it is presumed that

infants who have clinically improved with an FP are

better able to breath through their narrow trachea as long

as it is kept clear of stomach contents or not compressed

by a full esophagus. It is difficult to conclude which

operation these patients should have first to eliminate

their symptoms.

This paper has demonstrated in a retrospective fashion that

regardless of which cause is felt to be the major reason for the

respiratory distress, the surgeon must realize that either

operation (TM procedure or FP) alone may not be successful

in 33% to 46% of the cases necessitating a second type of

procedure. If the second procedure is indicated, it can be

expected to result in clinical improvement; all of the patients

in this study had resolution of their symptoms with the

second procedure.

References

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