The Long and Short of Esophageal Atresia

Transcription

Aleksandra Olszewski, MSIII
Gillian Lieberman, MD
The long and short of
esophageal atresia
Aleksandra Olszewski,
Harvard Medical School Year III
BIDMC Radiology Core Clerkship
August 2013
Outline
•
•
•
•
•
What is esophageal atresia (EA)?
Diagnosing EA: the role for imaging
Management of short-gap vs long-gap EA
The Foker Method
One patient’s story through her diagnosis,
therapy, complications, and cure
2
EA: Classification
-TEF
(8%)
Proximal
(1%)
+TEF
Both
(3%)
Distal
(84%)
*TEF without EA
(H type, 4%)
1. Pinheiro et al. World Journal of Gastroenterology 2012
3
July 28; 18(28): 3662-3672.
EA: the basics
• Most common esophageal abnormality (1/2500-1/3500)
• Cause: unclear
• Associated with anomalies >50% of the time
– Highest risk of VACTERL with isolated EA (no TEF)
• Presentation: at birth with excessive salivation,
suffocation, cyanosis, feeding intolerance, pneumonia
• Treatment: repaired surgically
2. Spitz L. Orphanet J Rare Dis 2007; 2: 24
3. Kovesi T, Rubin S. Chest 2004; 126: 915-925
4. Holland AJ, Fitzgerald DA. Paediatr Respir Rev 2010; 11: 100-106; quiz 106-107
4
EA: Prenatal diagnosis
• Prenatal: difficult to diagnose
– US: polyhydramnios + dilated proximal pouch
+ absence of gastric bubble
– not specific or sensitive findings, not always
present with TEF
– MR: nonvisualization of intra-thoracic
esophagus fundus
4. Holland AJ, Fitzgerald DA. Paediatr Respir Rev 2010; 11: 100-106; quiz 106-107
5. Houben CH, Curry JI. Prenat Diagn 2008; 28: 667-675
5
Companion Patient 1: AIUM (ACOG+ACR)
Guidelines for Obstetric Ultrasound Exams
Second/third trimester
screen: evaluation of
fetal presentation,
amniotic fluid volume,
cardiac activity,
placental position, fetal
biometry, and fetal
number, plus an
anatomic survey
(gastric bubble)
6. Mourali et al. La tunisie Medicale 2011 89(2):213-214.
6
7. AIUM 2012 Practice Guidelines for the Performance of Obstetric Ultrasound Exams
EA: Post-natal diagnosis
Delivery room:
– Symptoms, impassibility of orogastric catheter
past 11-12cm
– AP CXR with air as contrast (avoid aspiration of
contrast fluid!): catheter coiled in esophagus
• Air in stomach, indicates TEF  increased urgency
for surgery, high risk of aspiration
• Absence of air in stomach indicates no TEF
7
Companion Patients 2,3: EA with and
without TEF on AP Chest X-Ray
*
Left: note
nonprogression of
orogastric catheter,
lack of gastric air
bubble
*
8. LearningRadiology.com
9. radiopaedia.org
*
*
Right: note
nonprogression of
catheter, presence
of gastric air
bubble indicating
TEF
8
EA: Treatment
Pre-op: full aspiration precautions,
VACTERL assessment
-orogastric tube (suction), head of
bed 45°, acid suppression, avoid
PPV
-renal US, ECHO, etc.
Primary repair: thoracoscopic or
thoracotic ligation
-Contraindications:
2. Spitz L. Orphanet Journal of Rare Diseases. 2, 24. 2007.
Low birth weight, congenital
heart disease, long gap length,
compromised physiologic status
9
1. Pinheiro et al. World Journal of Gastroenterology 2012 July 28; 18(28): 3662-3672.
EA: Recognizing post-operative
complications through imaging
• Seven days post-op, esophogram is performed
• Leak  15% of cases
• Strictures  common, recurrent, tx: serial endoscopic
dilation
• GERD  common
• Esophageal dysmotility  75%-100% of cases
• Thoracic wall deformities  24% winged scapula, 21%
scoliosis
2. Spitz L. Orphanet J Rare Dis 2007; 2: 24
10. Mortell AE, Azizkhan RG. Semin Pediatr Surg 2009; 18:12-19.
10
Long-Gap EA: Management
• Primary surgery not possible
• Gapogram to assess gap size
– Treatment is controversial
Definition: gap
too big to repair
(>3cm or 2
vertebral bodies)
• Esophagus grows from swallowing (proximal) and reflux
(distal)  repair delay until 1-3 months
• Other options: replacement vs circular myotomy vs
surgery under tension vs Foker method
11. Bagolan et al. Diseases of the Esophagus 2013; 26: 372-379.
11
Companion patient 4: Gapogram to
assess gap size
• Contrast through enteric tube
to assess proximal stump
(then removed by enteric
tube)
• Contrast (or probe) through
gastrostomy to assess distal
stump
• Measure gap by vertebral
bodies or cm
12
12. Greenfield's Surgery: Scientific Principles and Practice.
Long-gap EA: Controversy
surrounding repair
OPTIONS
Growth
induction
Compiled information from:
13. Kunisaki SM, Foker JE. Clin Perinatol
39 (2012) 349–361
14. Spitz L. J Pediatr Surg
2006;41(10):1635–40.
15. Tsai JY et al. Ann Thorac Surg
1997;64(3):778–83.
Stomach
(gastric pull
up or gastric
tube)
Replacement
jejunum
colon
13
Long-gap EA: Management by Growth
Induction (the Foker process)
• Sutures applied through
external proximal and distal
stumps, with tag on end
• Sutures are pulled out of
patient’s body attached to
external traction devices
• With the patient paralyzed,
increasing traction is applied ove
time
14
16. Foker et al. Seminars in Pediatric Surgery 2005; 14:8-15.
Companion patient 5: Serial gapograms
show decreasing gap size over time in a
patient undergoing the Foker process
• Over time, traction causes stress leading to natural tissue growth
(rather than stretch, which may compromise tissue integrity)
• Serial gapograms are a critical part of the procedure, assessing
gap size in preparation for repair
16. Foker et al. Seminars in Pediatric Surgery 2005; 14:8-15.
15
Now that we know about
different types of EA, how to
diagnose each by imaging, and
how imaging is used with some
of the management options,
let’s meet our patient!
16
First, let’s go through our patient’s
diagnostic studies
17
Our Patient: AP Chest X-Ray at delivery
Pause to
evaluate
and
continue
for labeled
findings.
CHB PACS
18
Our Patient: Impassibility of NG tube
on AP Chest X-Ray at delivery
NG tube
impassible
proximally
Did you
remember to
assess for EA
AND for TEF?
This is a key
distinction that
changes the
management of
patients with EA.
*
Paucity of
gastric gas,
indicating
isolated EA
(without TEF)
19
CHB PACS
Our patient: Presentation and
Diagnosis
• GT is a 50-day-old ex-32-weeker who was
diagnosed prenatally with isolated EA (no
TEF).
• Postnatally, a diagnosis of EA without TEF was
confirmed, as you saw on chest X-Ray done at
delivery.
• She lives in Tennessee, and was transferred
to Boston Children’s for definitive care
20
Our Patient: Pre-operative evaluation
Two major consideration:
• Aspiration precautions
• VACTERL assessment
– She showed no signs of any other anomalies
(normal ECHO, no other GI/anal anomaly, no
limb anomalies, no vertebral anomalies,
normal kidneys)
21
Our Patient: Gapogram showing
34 mm gap size
*
*
Water-soluble barium gapogram
with contrast injected into the
enteric tube proximally and
contrast injected into a catheter
passed through the gastric tube
distally. A 34 mm gap between
distal and proximal esophageal
stumps was visualized. Note the
ruler behind the patient, to
calibrate findings.
22
CHB PACS
Our Patient: Imaging safety
considerations
Remember:
-Use water-soluble contrast in case of leak.
-Remove contrast from upper esophageal
pouch immediately after! These patients
have a blind-ending esophagus and it is
important to be prepared with suction
after imaging is performed to avoid
aspiration.
23
Our Patient: Management
• As seen on gapogram, her gap size is 34 mm
(dx: long-gap EA)
• She came to Children’s for the Foker process
• Her Foker 1 process (thoracotomy with
placement of sutures and traction devices) was
uncomplicated, and the ensuing slides follow
the serial gapograms that assessed her stump
approximation for esophageal anastomosis
24
Our patient, day 1 status post Foker 1 process: External
traction devices and esophageal stump tags on portable
supine chest x-ray
ET tube
enteral
tube
PICC
POD1 after Foker 1 process. Note the
external traction devices attached to
sutures at each esophageal stump.
The proximal and distal stumps are
tagged with markers. Barium
gapogram is needed for definitive
gap size assessment.
CHB PACS
25
Our patient day 5: External traction devices with
spacers and esophageal stump tags on portable
supine chest x-ray
Note the external traction
devices, now with spacers
to increase the tension on
the stumps. The proximal
and distal stumps are
tagged with markers, and
the distance between is
measured at 11.63mm
(need gapogram for
accurate assessment).
26
CHB PACS
Our patient day 5: Injection of contrast
proximally and distally on barium gapogram
*
*
Water soluble barium
injected through
enteric tube proximally
and through catheter
in the gastric tube
distally. At this point in
time, the proximal
esophagus and distally
the stomach are
visualized.
27
Our patient day 5: High attenuation contrast
extravasation on barium gapogram
In this next image
from the same study,
the upper
esophageal pouch
appears intact, but it
is evident that highattenuation contrast
has extravasated
from the distal
gastric catheter into
the pleural space,
indicating leak.
*
*
CHB PACS
28
After this, a repeat
thoracotomy was performed
and the ruptured distal
esophageal stump was
repaired.
29
Our patient day 30: Close approximation of proximal
and distal esophageal stumps on barium gapogram
*
*
Water soluble barium study highlighting approximation of
proximal and distal stumps, no
extravasation of contrast.
With the stumps wellapproximated, thoracotomy with
esophageal anastomosis was next
performed.
30
CHB PACS
Our patient post-operative day 1: Passage of
NG tube to stomach on supine chest x-ray
CHB PACS
31
Our patient post-operative
day 1: Evidence of stricture
formation on barium
esophophagram
*
CHB PACS
32
Companion patients 6, 7:
Strictures on barium esophagrams
CHB PACS
10-year-old female with chicken stuck in
esophagus due to stricture.
30-day-old male with evidence of
stricture.
33
Summary
We have seen:
• Key imaging signs to diagnose EA +/- TEF, prenatally and postnatally
• Imaging as it is used to evaluate gap size in EA (gapograms)
• How the Foker process uses serial gapograms to evaluate progress
• Imaging of complications in EA, including leak and strictures
We have discussed:
• Key considerations in diagnosis and management of long and short
gap EA, including safety precautions and pre-operative planning
• Different treatment options exist for long-gap EA
• Complications are common, but rarely severe
34
Thank you!
Yuri Shif, MD for his generous offering of time and
his assistance with image acquisition
Gillian Lieberman, MD, for her excellent teaching
and for directing our core radiology clerkship
Claire Odom for her organization and support
throughout the core radiology clerkship
35
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
Fernando P, Pinheiro M, Simoes e Silva AC, Pereira RM . Current knowledge on esophageal atresia.
World Journal of Gastroenterology 2012 July 28; 18(28): 3662-3672.
Spitz L. Oesophageal atresia. Orphanet J Rare Dis 2007; 2: 24.
Kovesi T, Rubin S. Long-term complications of congenital esophageal atresia and/or tracheoesophageal
fistula. Chest 2004; 126: 915-925.
Holland AJ, Fitzgerald DA. Oesophageal atresia and tracheo-oesophageal fistula: current management
strategiesand complications. Paediatr Respir Rev 2010; 11: 100-106; quiz 106-107.
Houben CH, Curry JI. Current status of prenatal diagnosis, operative management and outcome of
esophageal atresia/tracheo-esophageal fistula. Prenat Diagn 2008; 28: 667-675.
Mourali M, Essoussi-Chikhaoui J, Fatnassi A, El Fekih C, Ghorbel S, Ben Zineb N, Oueslati B. Prenatal
diagnosis of esophageal atresia. La tunisie Medicale 2011; 89(2):213-214.
American Institute of Ultrasound in Medicine. AIUM practice guideline for the performance of obstetric
ultrasound examinations. J Ultrasound Med 2013; 32: 1083–1101. doi:10.7863/ultra.32.6.1083LR
Learning Radiology case of the week. Esophageal atresia and tracheoesophageal atresia.
LearningRadiology.com 2005.
Alsalam H. Oesophageal atresia with trache-oesophageal fistula. Radiopaedia Cases. Radiopaedia.org.
Mortell AE, Azizkhan RG. Esophageal atresia repair with thoracotomy: the Cincinnati contemporary
experience. Semin Pediatr Surg 2009; 18:12-19.
Bagolan P., Valfre L, Morini F, Conforti A. Long-gap esophageal atresia: traction-growth and
36
anastomosis-before and beyond. Diseases of the Esophagus 2013; 26:372-379.
References
12. Mulholloand MW, Lillemoe KD, Doherty GM, Maier RV, Simeone DM, Upchurch GR. Greenfield's Surgery:
Scientific Principles and Practice, 5e > Chapter 106. The Pediatric Chest. Lippincott Williams & Wilkins, Sept 11 2012.
13. Kunisaki SM, Foker JE. Surgical advances in the fetus and neonate: esophageal atresia. Clin Perinatol 2012; 39:
349–361.
14. Spitz L. Esophageal atresia. Lessons I have learned in a 40-year experience. J Pediatr Surg 2006; 41(10):1635–40.
15.Tsai JY, Berkery L, Wesson DE, Redo SF, Spigland NA. Esophageal atresia and tracheoesophageal fistula: surgical
experience over two decades. Ann Thorac Surg 1997; 64(3): 778–83.
16.Foker JE, Kendall TC, Catton K, Khan KM. A flexible approach to achieve a true primary repair for all infants with
esophageal atresia. Seminars in Pediatric Surgery 2005; 14: 8-15.
37

The Long and Short of Esophageal Atresia

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