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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: mliszewski@univrad.com                                          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-
References                                                                          gist should know. Acta Radiol 54:221–225
                                                                                 9. Spitz L (2007) Oesophageal atresia. Orphanet J Rare Dis 2:24
   1. David TJ, O'Callaghan SE (1975) Oesophageal atresia in the South          10. Seguier-Lipszyc E, Bonnard A, Aizenfisz S et al (2005) The
      West of England. J Med Genet 12:1–11                                          management of long gap esophageal atresia. J Pediatr Surg
   2. Ein SH, Shandling B (1994) Pure esophageal atresia: a 50-year                 40:1542–1546
      review. J Pediatr Surg 29:1208–1211                                       11. Best C, Sudel B, Foker JE et al (2009) Esophageal stenting in
   3. Spitz L (1996) Esophageal atresia: past, present, and future. J Pediatr       children: indications, application, effectiveness, and complications.
      Surg 31:19–25                                                                 Gastrointest Endosc 70:1248–1253
   4. Vogel AM, Yang EY, Fishman SJ (2006) Hydrostatic stretch-induced          12. Kramer RE, Quiros JA (2010) Esophageal stents for severe strictures
      growth facilitating primary anastomosis in long-gap esophageal atre-          in young children: experience, benefits, and risk. Curr Gastroenterol
      sia. J Pediatr Surg 41:1170–1172                                              Rep 12:203–210
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