Figure 1
Anteroposterior chest radiograph
Anteroposterior chest radiograph. The distal part of the nasogastric tube is looped in the proximal oesophagus (arrow), while there is air in the stomach (asterisk).
Oesophageal atresia type C
SectionPaediatric radiology
Case TypeClinical Cases
AuthorsVassalou Evangelia, Grammatikakis Ioannis, Raissaki Maria, Karantanas Apostolos
Connected authors
2 days, female
Clinical History
A two-day-old, full term, female neonate presented with difficulty in feeding, multiple regurgitations and excessive oral secretions, since birth. The child was born by uncomplicated vaginal delivery. Clinical examination was unremarkable and routine blood tests were normal.
Imaging Findings
A nasogastric (NG) tube was inserted which could not pass beyond 10 cm. Chest radiograph showed the distal NG tube looped in the proximal 1/3 of thoracic oesophagus and multiple air-filled bowel loops at the upper abdomen (Fig. 1). A contrast study was requested to exclude a proximal tracheo-oesophageal fistula. Following administration of 10 ml of nonionic, water-soluble contrast material through the tube, under fluoroscopic control, there was pooling of the contrast at the blindly-ending distended proximal oesophagus (Fig. 2). A few seconds later, bubbly-appearing contrast was present in the proximal trachea, from the level of the cricopharyngeal muscle, to the right proximal bronchial tree (Fig. 3). The remaining contrast was immediately retracted through the NG tube. The neonate was referred for an upper gastro-intestinal tract examination, and shortly after for surgical repair. Free passage of contrast was noted through the oesophagus (Fig. 4) while multiple episodes of gastro-oesophageal reflux were recorded.
Discussion
Background
Oesophageal atresia (EA) is loss of continuity between the upper and lower oesophagus, representing its commonest congenital malformation. This developmental disorder results from failure of the primitive foregut to divide into trachea anteriorly and oesophagus posteriorly [1].
The estimated incidence is 1:3500 live births [2].
An associated tracheo-oesophageal fistula (TEF) shows five subtypes [3]:
1. Type A: EA without TEF (7%)
2. Type B: EA with proximal TEF (2%)
3. Type C: EA with distal TEF (86%)
4. Type D: EA with proximal and distal TEF (1%)
5. Type E: TEF without EA (H-fistula) (4%)
Up to 60% of individuals with EA/TEF have additional congenital anomalies in the following settings [2]:
1. Isolated: Absence of associated congenital anomalies.
2. Syndromic: Associated with other congenital defects under the context of a characterized genetic aetiology.
3. Non-isolated, non syndromic: Associated with other defects, usually conforming to the acronym VACTERL (Vertebral disorders, Ano-rectal malformations, Cardiovascular anomalies, TEF, Radial/Renal dysplasia, Limb defects) [4].
Clinical Perspective
Clinical features include choking, coughing, inability to swallow, regurgitations and aspiration during early feedings. Presentation is typically early, except for type E which may remain undiagnosed until childhood [1].
Imaging Perspective
In all types excluding type E, chest X-ray shows the NG tube looped in the proximal oesophageal pocket. Air in the stomach is indicative of a distal TEF (types C/D), while a gasless stomach excludes a distal TEF (types A/B). A proximal TEF may be revealed fluoroscopically with the use of a small amount of nonionic contrast material [1]. The diagnosis of isolated H-fistula requires a modified fluoroscopic study with the patient prone and a horizontal X-ray beam, in order to depict the TEF which passes obliquely and superiorly from the oesophagus to the trachea [5].
Outcome
Primary surgical correction of EA and TEF is the optional treatment in the absence of severe malformations.
Abnormal findings postoperatively include leak, narrowing of the anastomosis, gastroesophageal reflux, oesophageal dysmotility, fistula recurrence. Tracheomalacia and respiratory disorders may also occur [6].
Take Home Message, Teaching Points
EA is diagnosed when a looped NG tube is noted within the dilated proximal oesophagus. The presence of air in the stomach is indicative of a distal TEF (types C/D), while a gasless stomach excludes a distal TEF (types A/B). The diagnosis of type E malformation, usually delayed, is suggested by the depiction of H-type fistula, preferably during a modified fluoroscopic study.
Oesophageal atresia (EA) is loss of continuity between the upper and lower oesophagus, representing its commonest congenital malformation. This developmental disorder results from failure of the primitive foregut to divide into trachea anteriorly and oesophagus posteriorly [1].
The estimated incidence is 1:3500 live births [2].
An associated tracheo-oesophageal fistula (TEF) shows five subtypes [3]:
1. Type A: EA without TEF (7%)
2. Type B: EA with proximal TEF (2%)
3. Type C: EA with distal TEF (86%)
4. Type D: EA with proximal and distal TEF (1%)
5. Type E: TEF without EA (H-fistula) (4%)
Up to 60% of individuals with EA/TEF have additional congenital anomalies in the following settings [2]:
1. Isolated: Absence of associated congenital anomalies.
2. Syndromic: Associated with other congenital defects under the context of a characterized genetic aetiology.
3. Non-isolated, non syndromic: Associated with other defects, usually conforming to the acronym VACTERL (Vertebral disorders, Ano-rectal malformations, Cardiovascular anomalies, TEF, Radial/Renal dysplasia, Limb defects) [4].
Clinical Perspective
Clinical features include choking, coughing, inability to swallow, regurgitations and aspiration during early feedings. Presentation is typically early, except for type E which may remain undiagnosed until childhood [1].
Imaging Perspective
In all types excluding type E, chest X-ray shows the NG tube looped in the proximal oesophageal pocket. Air in the stomach is indicative of a distal TEF (types C/D), while a gasless stomach excludes a distal TEF (types A/B). A proximal TEF may be revealed fluoroscopically with the use of a small amount of nonionic contrast material [1]. The diagnosis of isolated H-fistula requires a modified fluoroscopic study with the patient prone and a horizontal X-ray beam, in order to depict the TEF which passes obliquely and superiorly from the oesophagus to the trachea [5].
Outcome
Primary surgical correction of EA and TEF is the optional treatment in the absence of severe malformations.
Abnormal findings postoperatively include leak, narrowing of the anastomosis, gastroesophageal reflux, oesophageal dysmotility, fistula recurrence. Tracheomalacia and respiratory disorders may also occur [6].
Take Home Message, Teaching Points
EA is diagnosed when a looped NG tube is noted within the dilated proximal oesophagus. The presence of air in the stomach is indicative of a distal TEF (types C/D), while a gasless stomach excludes a distal TEF (types A/B). The diagnosis of type E malformation, usually delayed, is suggested by the depiction of H-type fistula, preferably during a modified fluoroscopic study.
Differential Diagnosis List
Oesophageal atresia type C
none
none
Final Diagnosis
Oesophageal atresia type C
References
[1] Berrocal T, Madrid C, Novo S, Gutierrez J, Arjonilla A, Gomez-Leon N (2004) Congenital Anomalies of the Tracheobronchial Tree, Lung, and Mediastinum: Embryology, Radiology, and Pathology. Radiographics 24:e17 (PMID: 14610245)
[2] Shaw-Smith CJ (2006) Oesophageal atresia, tracheo-oesophageal fistula, and the VACTERL association: review of genetics and epidemiology. J Med Genet 43:545–54 (PMID: 16299066)
[3] Gross RE (1953) The surgery of infancy and chilhood. Philadelphia, PA: WB Saunders p:76
[4] Chittmittrapap S, Spitz L, Kiely EM, Brereton RJ (1989) Oesophageal atresia and associated anomalies. Arch Dis Child 64:364–368 (PMID: 1791901)
[5] Hiorns MP (2011) Gastrointestinal tract imaging in children: current techniques. Pediatr Radiol 41:42-54 (PMID: 20596703)
[6] Mortell AE, Azizkhan RG (2009) Esophageal atresia repair with thoracotomy: the Cincinnati contemporary experience. Semin Pediatr Surg 18:12-19 (PMID: 19103416)
Case information
| URL: | https://www.eurorad.org/case/11971 |
| DOI: | 10.1594/EURORAD/CASE.11971 |
| ISSN: | 1563-4086 |
Figure 2
Fluoroscopic study, right oblique view
There is contrast pooling in the proximal oesophagus and evidence of aspiration with contrast in the trachea (asterisk), from the level of cricopharyngeal muscle, to the right bronchial tree (arrow). No evidence of proximal fistula.
Figure 3
Postoperative contrast study
Postoperative fluoroscopic image showing free passage of contrast through the oesophagus to the upper gastro-intestinal tract.
Figure 4
Fluoroscopic study, right oblique view
Fluoroscopic study, right oblique view. There is pooling of contrast material (asterisk) in the blindly-ending mildly distended proximal oesophagus and contrast in the valleculae.
Anteroposterior chest radiograph
Anteroposterior chest radiograph. The distal part of the nasogastric tube is looped in the proximal oesophagus (arrow), while there is air in the stomach (asterisk).
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Fluoroscopic study, right oblique view
There is contrast pooling in the proximal oesophagus and evidence of aspiration with contrast in the trachea (asterisk), from the level of cricopharyngeal muscle, to the right bronchial tree (arrow). No evidence of proximal fistula.
Unknown
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Postoperative contrast study
Postoperative fluoroscopic image showing free passage of contrast through the oesophagus to the upper gastro-intestinal tract.
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Unknown
Fluoroscopic study, right oblique view
Fluoroscopic study, right oblique view. There is pooling of contrast material (asterisk) in the blindly-ending mildly distended proximal oesophagus and contrast in the valleculae.
Unknown
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