imaging of congenital - international foundation for pediatric … of congenital disorders of... ·...
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![Page 1: Imaging of congenital - International Foundation for Pediatric … of congenital disorders of... · Imaging of congenital disorders of esophagus in infancy and childhood M. Mearadji](https://reader031.vdocument.in/reader031/viewer/2022011809/5d037b9388c993b06c8e2762/html5/thumbnails/1.jpg)
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Imaging of congenital
disorders of esophagus in
infancy and childhood
M. Mearadji
International Foundation for Pediatric Imaging Aid
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Introduction
• From practical point of view the esophageal abnormalities have to be divided into congenital and acquired diseases
• Congenital diseases manifest themselves mostly in early life
• Acquired disorders are usually expected in later life
• Each group of abnormalities should be categorized etiologically
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Congenital disorders of
esophagus
1. Esophageal atresia (EA) and tracheo-esophageal fistula
2. Congenital esophageal stenosis
3. Esophageal duplication
4. Laryngo-tracheo-esophageal cleft (LTE)
5. Diverticula
6. Epidermolysis bullosa dystrophica
7. Extrinsic impression of esophagus by vascular ring or mediastinal masses
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Esophageal atresia (EA) • Frequency 1 : 2500-3000 life births
• Exact etiology unknown, but it is a developmental disorder in formation and separation of the primitive foregut into trachea and esophagus
• Inability to swallow salivary secretions with overflow and aspiration
• Various types of esophageal atresia
7% ±1
%
86% 2% 4%
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Associated anomalies
(VACTERL) with EA and TEF
VACTERL association is an acronym for a complex of
anomalies affecting various systems
– V = Vertebral
– A = Anorectal
– C = Cardiovascular
– TE = Tracheo-esophageal
– R = Renal
– L = Limbs
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Preoperative imaging of EA
• Positioning of a radio-opaque feeding tube to the level of atretic pouch
• Cervicothoraco-abdominal plain film (AP), if necessary added with a lateral chest film
• Contrast pouchogram of atretic proximal esophagus is unnecessary because of risk of aspiration
• Use of non-ionic contrast medium is absolutely indicated in only suspected cases of TEF without atresia (H-type)
• Cardiac and abdominal sonography
• CT and MRI are only useful in exceptional cases
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Radiological findings
• The radiological appearance varies with the type of lesion
• The proximal blind ending pouch is usually lucent and distended with air
• In cases with a distal TEF bowel gas is present
• A gasless abdomen is characteristic for EA without distal fistula (long gap esophageal atresia)
• Special attention should be paid in recognition of other GI tract obstruction, cardiopulmonary and skeletal abnormalities
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Aspiration pneumonia after use of
contrast medium by EA
Neonate with esophageal
atresia and right-sided
pulmonal agenesis.
Note the dilated proximal
esophageal pouch with air.
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Two cases of tracheo-esophageal fistula without atresia
Non-ionic contrast media has been used
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Postoperative findings and
complication of EA
• Gastro-esophageal reflux (70-100%)
• Esophageal stricture (up to 40%) specially following leakage
• Esophageal leak ± 18%
• Tracheomalacia
• Poor esophageal motility and lodging of large bolus of food above the anastomosis
• Recurrent fistula
• Additional lower congenital stenosis
• Incidental finding of a overlooked proximal fistula
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Reflux Stricture
Tracheo-
malacia Leakage
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Recurrent
TEF
Proximal fistula years after
primary correction
Lodging of
food
Second
congenital
stenosis by EA
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EA without distal fistula
Long gap (atresia)
A: primary correction
B: colonic interposition
C: jejunal interposition
B A C
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Congenital esophageal stenosis
• Frequency 1 : 25.000-50.000 life birth
• One third of cases are associated with EA
• Located mostly in distal third of the esophagus
• Some stenoses are membranous web shaped such as diaphragm and can be multiple
• They may occur as an isolated narrowing due to ectopic cartilagineous tracheobronchial remnants; some other due to segmental hypertrophy of muscular layers or fibrosis of mucosal layers
• The stenosis should be differentiated from peptic stenosis related to gastro-oesophageal reflux disease
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Note the different
types of one or
more congenital
webs and stenoses
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Esophageal duplication cysts
• Related to an abnormality in tubulation of esophagus
• Second most common duplication after ileal duplication (15-20%)
• Usually no communication with esophageal lumen
• Most common incidental finding on chest film or by contrast study of GI tract
• Additionally to contrast esophagogram, US, CT and MRI are all helpful to demonstrate the location, extension and its relation to neighbouring organs
• Duplication cysts should be differentiated from other cystic or solid mediastinal masses, especially from bronchogenic cysts
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Two cases of duplication cyst of esophagus
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Laryngotracheo-esophageal cleft
(LTE)
• Very rare congenital abnormality
• Depending to its extension classified in four types
• Majority has associated congenital malformations such as esophageal atresia, congenital heart diseases and pulmonary abnormalities
• Clinical findings: cyanosis, choking on feeding and aspiration
• Contrast examination: free spillage of contrast from esophagus to trachea (should be avoided)
• CT is a possibility to demonstrate the LTE cleft
• Endoscopic evaluation is the first method of choice for diagnosis and classification of the LTE cleft
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Type I Type II
Two cases of laryngothracheoesophageal cleft
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Diverticula
• Rarely to be observed in children
• Pressure diverticula are herniations of the mucosa and submucosa through congenitally weak sites of the esophagus wall
• Usually located above the clavicles
• Best visible in lateral or oblique projection
• Large diverticula can be observed following leak of repaired esophageal atresia and should be differentiated from congenital diverticula
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Congenital diverticula located in
upper part of the esophagus
Large diverticula following
leakage of corrected
esophageal atresia (acquired)
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Epidermolysis bullosa
dystrophica
• A congenital disease affecting squamous epithelium with involvement of esophagus
• Inherited in an autosomal recessive pattern
• Can produce strictures and dysmotility of esophagus
• Gastro-intestinal manifestation can occur in the absence of cutaneous changes
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A case of epidermolysis bullosa
dystrophica with esophageal
stricture
Another case of
epidermolysis
bullosa dystrophica
with atretic
esophagus and
pyloric canal
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Vascular rings anomalies
• Extrinsic impressions on the esophagus
• The main complaints are related to the airways compression and rarely lead to dysphagia
• An anterior compression on the trachea with a posterior impression on the esophagus is a vascular ring
• A vascular ring is a result of either a double aortic arch or a right aortic arch with an aberrant left subclavicular artery and ductus arteriosus remnant
• Additionally to initial esophagram, CTA or MRA are absolutely indicated in preoperative deliniation of the anatomy of the vascular ring
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Imaging of arcus aortae duplex
with extrinsic esophageal
compression