Imaging LGEA

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Imaging of long gap esophageal atresia and the Foker process: Expected
findings and complications

Article  in  Pediatric Radiology · December 2013


DOI: 10.1007/s00247-013-2847-2 · Source: PubMed

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Pediatr Radiol (2014) 44:467–475
DOI 10.1007/s00247-013-2847-2

PICTORIAL ESSAY

Imaging of long gap esophageal atresia and the Foker


process: expected findings and complications
Mark C. Liszewski & Sigrid Bairdain & Carlo Buonomo &
Russell W. Jennings & George A. Taylor

Received: 24 June 2013 / Revised: 13 October 2013 / Accepted: 21 November 2013 / Published online: 24 December 2013
# Springer-Verlag Berlin Heidelberg 2013

Abstract Long gap esophageal atresia (EA) is characterized Introduction


by esophageal segments that are too far apart for primary
anastomosis. Surgical repair utilizing interposition grafts or Esophageal atresia (EA) with or without tracheoesophageal
gastric transposition are often employed. The Foker staged fistula (TEF) is a rare congenital disorder, occurring in ap-
lengthening procedure is an alternative surgical method that proximately 1:4,500 live births [1]. The term long gap esoph-
utilizes continuous traction on the esophagus to induce esoph- ageal atresia is applied when the distance between the upper
ageal growth and allow for primary esophageal anastomosis. and lower atretic segments is too far for primary anastomosis.
This pictorial review presents the step-by-step radiographic Because primary anastomosis is not possible in this group of
evaluation of the Foker procedure and illustrates the radio- patients, several surgical techniques are utilized to establish
graphic findings in the most commonly encountered compli- continuity between the atretic segments. These include 1)
cations in our cohort of 38 patients managed with this proce- primary repair under tension; 2) gastric mobilization with
dure from January 2000 to June 2012. partial gastric pull up; 3) lengthening myotomies; 4) proximal
and distal pouch stretching by various methods, and 5)
Keywords Long gap esophageal atresia . Foker process . esophageal replacement with gastric, colon or jejunum
Children . Esophagus . Esophagram interpositions [2–4]. In 1997, Foker et al. [5] described
an alternative technique that utilizes traction sutures to
promote in vivo esophageal growth through tension-
induced lengthening and subsequent delayed primary repair.
M. C. Liszewski : C. Buonomo : G. A. Taylor
Therefore, the Foker process allows for the native esophagus
Department of Radiology, Boston Children’s Hospital, to serve as the conduit, even when the atretic segments are
Harvard Medical School, Boston, MA, USA widely spaced.
The Foker process is a two-stage surgical procedure. Stage
M. C. Liszewski (*)
I consists of placing the esophageal segments under traction
Rutgers Robert Wood Johnson Medical School,
Department of Radiology, Robert Wood Johnson University Hospital, (Fig. 1). Stage II is the esophageal anastomosis after traction-
One Robert Wood Johnson Place, New Brunswick, NJ 08903, USA induced growth. As with all EA patients, there is a high
e-mail: [email protected] incidence of gastroesophageal reflux (GER) in these children
S. Bairdain : R. W. Jennings
and a large number of patients subsequently go on to gastric
Department of Surgery, Boston Children’s Hospital, fundoplication. It is important that radiologists are familiar
Harvard Medical School, Boston, MA, USA with the expected radiographic findings at each stage as well
468 Pediatr Radiol (2014) 44:467–475

Fig. 1 Foker Stage I. a


Schematic demonstrates
esophageal segments marked
by radiopaque clips (black
arrowhead) and attached to
lengthening apparatuses in the
chest wall (black arrow) with
traction sutures (white arrow).
b Intraoperative photograph
demonstrates an upper
esophageal segment (black
arrow) with traction sutures in
place (white arrow)

as commonly encountered complications in this patient group which requires access via a percutaneous gastrostomy. If
[6–8]. This Institutional Review Board-approved pictorial a gastrostomy is present, patients are brought to the
essay will highlight the imaging findings encountered in our fluoroscopy suite and a nasoesophageal catheter is po-
cohort of 38 children managed with this procedure from sitioned with its tip in the proximal esophageal segment
January 2000 to June 2012 and will outline what radiologists and a second catheter is positioned with its tip in the
need to know. distal segment via a gastrostomy. The gap length is
determined by injecting a water-soluble contrast agent
such as ioversol (Optiray-300; Mallinckrodt, St. Louis,
Imaging prior to surgery MO, USA) and referencing a calibrated ruler placed in
the image field (Fig. 3). If there is reflux into the distal
Plain radiographs are frequently the first imaging test obtained esophageal segment, positioning the distal catheter in
in this patient group. After birth, initial radiographs may the stomach is often sufficient. In our institution the surgical
demonstrate a dilated upper esophageal segment or a team is present during the esophagram and places the
nasoenteric tube terminating in the upper esophageal segment. enteric tubes. We use 5-Fr catheters. If gastrostomy is
Eighty-five percent will have a distal TEF and air in the not present, the initial “gap-o-gram” esophagram may be
GI tract and 5% will have pure EA with no fistula and performed in the operating room at the time of gastrostomy
the abdomen will be gasless (Fig. 2). EA is associated placement.
with additional anomalies in approximately 50% of cases,
the majority involving one or more of the VACTERL associ-
ation (vertebral, anorectal, cardiac, tracheoesophageal, Imaging after traction suture placement (Stage I)
radial ray/renal and limb anomalies) [9]. Therefore, par-
ticular attention should be paid to the presence of associated Radiographic evaluation after traction suture placement depends
anomalies when interpreting radiographs of patients with largely on the clinical situation. All patients receive frequent
EA. chest radiographs. Additional studies may include follow-up
Once the diagnosis of EA is established, the length of the esophagram, US and/or CT as clinically indicated. In the preop-
gap is evaluated by performing a “gap-o-gram” esophagram, erative period, care must be taken when placing the enteric tubes
Pediatr Radiol (2014) 44:467–475 469

Fig. 3 Presurgical “gap-o-gram” esophagram in a 2-day-old boy with


esophageal atresia. Intraoperative “gap-o-gram” esophagram with a cal-
ibrated ruler demonstrates a 5.5-cm gap between the proximal (black
arrow) and distal (white arrow) esophageal segments

Fig. 2 Frontal radiograph of the chest and abdomen in a newborn male


with esophageal atresia. The abdomen is gasless, a nasoenteric tube
terminates in an upper esophageal pouch (black arrow) and a lumbar
hemivertebra (white arrow) is noted

when performing “gap-o-gram” esophagram due to the fragility


of the esophageal pouches and risk of iatrogenic perforation.

Expected findings after Stage I

Stage I of the Foker process involves thoracotomy, placement


of surgical clips at the end of each esophageal segment to act
as radiographic markers and placement of traction sutures that
are externalized to lengthening apparatuses on the skin (Figs. 1
and 4). Tension is induced by periodically adding catheter
tubing to the lengthening apparatuses. Esophageal growth is
monitored on serial chest radiographs (Fig. 5) by tracking the
position of clips and on “gap-o-gram” esophagrams (Fig. 6)
by tracking the position of the opacified esophageal lumen.

Complications after Stage I Fig. 4 Chest radiograph in a 6-month-old boy after Stage I. The proximal
esophageal segment is marked by a clip (black arrowhead) and attached
to the lower lengthening apparatus (black arrow). The distal esophageal
Complications occurring after Stage I may include esophageal segment is also marked by a clip (white arrowhead) and attached to the
segment leak, empyema and/or abscess. Patients are sedated upper lengthening apparatus (white arrow)
470 Pediatr Radiol (2014) 44:467–475

Fig. 5 Serial chest radiographs in a 1-month-old boy undergoing the radiographs on postoperative day 1 (a), 5 (b) and 11 (c) demonstrate
Foker process demonstrate traction-induced esophageal growth. The ends increased catheter tubing, which provides continuous traction, and move-
of the esophageal segments are marked by clips (arrowheads) and the ment of the esophageal markers indicating tension-induced growth, with
traction devices consist of anchors and pieces of catheter tubing (arrows). several centimeters of overlap by postoperative day 11. Subsequent “gap-
The proximal esophageal segment (black arrowhead) is attached to the o-gram” esophagram (not shown) demonstrated intact overlapping
lower device (black arrow) and the distal esophageal segment (white esophageal segments
arrowhead ) is attached to the upper device (white arrow ). Chest

and paralyzed throughout Stage I, but paralysis is peri- Imaging after esophageal anastomosis (Stage II)
odically lifted and suture disruption may occur. If there
is disruption of an esophageal segment, extraluminal gas Expected findings after Stage II
and debris may be seen on radiographs and leak may be
confirmed on “gap-o-gram” esophagram (Fig. 7). Infec- Once adequate esophageal length is achieved patients un-
tions of the chest cavity, often related to leak, can lead dergo Stage II, consisting of repeat thoracotomy and
to empyema or abscess (Fig. 8). esophageal anastomosis. After anastomosis, an esophagram

Fig. 6 “Gap-o-gram”
esophagrams demonstrate
tension-induced growth of the
proximal (black arrowhead)
and distal (white arrowhead)
esophageal segments, occurring
between esophagram performed
on the day after transfer to our
institution (a) and after 14 days of
tension-induced growth (b)
Pediatr Radiol (2014) 44:467–475 471

Fig. 8 Contrast-enhanced CT of the chest in a 2-year-old boy with long


gap esophageal atresia undergoing the Foker process with known lower
segment leak, fever and leukocytosis. Axial (a) and coronal (b) images
demonstrate a peripherally enhancing empyema (black arrows) with
extension of phlegmon to the chest wall (white arrows)
Fig. 7 Pouch leak in a patient undergoing the Foker process. Contrast
medium is injected through catheters positioned in the proximal and distal
pouches, and leak (arrow) is seen arising from the proximal pouch complications [7, 10]. Strictures are often identified on
esophagram and are treated with balloon dilatation (Fig. 10).
Removable covered stents may be utilized in selected cases of
is performed by administering an oral feed or positioning recalcitrant stricture [11, 12] (Fig. 10). Leaks can be suggested
a nasoenteric tube within the proximal esophagus and by findings of new pleural fluid on chest radiograph or US and
injecting water-soluble contrast medium under fluoroscopic are confirmed on esophagram (Fig. 11). Leaks tend to occur at
observation (Fig. 9). Care must be taken to avoid the anastomosis and occur over a broad time interval with later
disrupting the fragile anastomosis if performing the leaks often occurring after stricture dilation. Like pouch leaks,
esophagram via a nasoenteric tube. In our institution, the anastomotic leaks predispose to empyema and abscess. Addi-
surgical team is present during the esophagram and posi- tional findings after Stage II include gastroesophageal reflux
tions the nasoenteric tube for the study. Contour irregular- (Fig. 12) and hiatal hernia (Fig. 13).
ity is often noted at the anastomotic site, but the esopha-
gus is expected to be widely patent without leak.
Imaging after gastric fundoplication
Complications after Stage II
Expected findings after fundoplication
The most common complications following Stage II are
esophageal stricture and leak. Surgically induced tension and There is a high incidence of GER in all patients with EA,
GER are both thought to increase the risk for these and when reflux occurs after anastomosis this may lead to
472 Pediatr Radiol (2014) 44:467–475

an increased incidence of strictures and leaks [7, 10]. Gastric


fundoplication is performed to help alleviate this. Imaging
after fundoplication begins with gastrostomy injection to
evaluate for gastroesophageal reflux. If no reflux is present,
the patient swallows contrast medium and esophagram is
performed. On esophagram, the fundoplication wrap is seen
(Fig. 14) and there should be timely antegrade passage of
contrast medium through the wrap without obstruction.

Complications after fundoplication

Complications after fundoplication include delayed transit


across the fundoplication and persistent gastroesophageal re-
flux (Figs. 15 and 16). Delayed transit may be related to
postoperative edema and resolve with time or due to a tight
configuration of the wrap requiring balloon dilatation or sur-
gical revision. Persistent gastroesophageal reflux often re-
quires surgical revision.

Osseous findings: fracture and chest wall deformity

Patients undergoing the Foker process for the treatment of


long gap EA have a high incidence of fractures. Fifty
percent of patients in our cohort sustained a long bone
Fig. 9 Satisfactory postoperative esophagram in a 7-month-old girl, fracture. Buckle-type and minimally displaced fractures are
postoperative day 13 after anastomosis. Nasoenteric tube is positioned most common, and they most frequently occur in the
within the proximal esophagus and water-soluble contrast medium is proximal humerus and distal femur. Prolonged paralysis
injected. Mild narrowing (arrow) at anastomosis is expected in the
immediate-postoperative period and fluid restriction lead to osseous demineralization and

Fig. 10 Esophageal stricture,


balloon dilatation and stent after
the Foker process. Esophagram
(a) on postoperative day 13
demonstrates mid-esophageal
stricture (black arrow). Balloon
dilatation (white arrow, b) was
performed several times to treat
the esophageal stricture. A
persistent stricture was treated
with a covered removable
esophageal stent (black
arrowhead). Esophagram (c)
demonstrates patency of the
esophagus through the stent
Pediatr Radiol (2014) 44:467–475 473

Fig. 11 A 15-month-old boy


with long gap EA undergoing the
Foker process, status post
esophageal anastomosis with
esophageal leak on postoperative
day 18. Chest radiograph (a)
demonstrates pleural fluid
(black arrow). Esophagram (b)
demonstrates a leak with
esophagopleural fistula
(white arrows). Ultrasound (c)
demonstrates pleural fluid
(white arrowhead). A 10-Fr
pleural pigtail catheter was
placed. Fluoroscopic image
obtained after nasoenteric
tube contrast injection (d)
demonstrates persistent leak
(black arrowheads)

Fig. 13 Esophagram in a 4-month-old boy undergoing the Foker process


Fig. 12 Gastrostomy port injection of a gastrojejunostomy tube in a 2- 53 days after anastomosis demonstrates a hiatal hernia (black arrow) and
month-old boy 14 days after anastomosis demonstrates GER narrowing at the anastomosis (white arrow)
474 Pediatr Radiol (2014) 44:467–475

Fig. 14 Esophagram after the Foker process and fundoplication for


treatment of GER demonstrates an expected filling defect from gastric Fig. 16 Gastrostomy contrast medium injection in a 12-month-old girl
fundoplication (arrow) postoperative day 8 after fundoplication demonstrates faint impression
from fundoplication (black arrow) and gastroesophageal reflux (white
arrow)

are thought to underlie this increased fracture risk. Repeat deformity (Fig. 17), and should be noted because of an
thoracotomies lead to varying degrees of chest wall increased risk of scoliosis later in life.

Fig. 15 Esophagram performed on postoperative day 6 after Fig. 17 Chest radiograph in a 2-year-old girl with long gap EA status
funcoplication demonstrates obstruction at the fundoplication (arrow) post Foker process demonstrates significant rib and chest wall deformities
with dilatation of the esophagus and no contrast medium passage to (arrow) and a chronic right upper lung opacity due to plural thickening
the stomach and parenchymal scar
Pediatr Radiol (2014) 44:467–475 475

Conclusion 5. Foker JE, Linden BC, Boyle EM Jr et al (1997) Development of a


true primary repair for the full spectrum of esophageal atresia. Ann
Surg 226:533–541, discussion 541-533
Radiology is integral to the management of long gap EA 6. Sri Paran T, Decaluwe D, Corbally M et al (2007) Long-
utilizing the Foker process. Familiarity with expected and term results of delayed primary anastomosis for pure oe-
unexpected imaging findings in this multistage procedure will sophageal atresia: a 27-year follow up. Pediatr Surg Int 23:
647–651
help the radiologist to provide optimum care for children.
7. Friedmacher F, Puri P (2012) Delayed primary anastomosis
for management of long-gap esophageal atresia: a meta-
analysis of complications and long-term outcome. Pediatr
Conflict of interest None. Surg Int 28:899–906
8. Sodhi KS, Saxena AK, Ahuja CK et al (2013) Postoperative
appearances of esophageal atresia repair: retrospective study
of 210 patients with review of literature—what the radiolo-
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