Korean Society of Thoracic Radiology
The Korean Radiological Society
History
A 69-year-old male heavy alcoholics presented with
swallowing difficulty, which aggravated after endoscopy at private clinic.
Findings
CXR: mediastinal widening
Initial Chest CT: - entire esophagus, 2 esophageal lumina, low
density(30 HU) lesion
Esophagogram: double-barreled esophagus
F/U (3 months) HRCT: 2 esophageal lumina
Discussion
Duplication of the alimentary tract are rare congenital
malformations that may occur at any level from mouth to the anus. The most
common site is ileum. Duplication of the esophagus is the second most common
duplication of the gastrointestinal tract, consisting 10-15% of those reported.
There are 2 types of esophageal duplications: cystic and tubular. Of 44
cases reviewed, only 6 cases were of the tubular variety(1). Although the
exact etiology of duplication of the espophagus has not been established,
several theories have been proposed such as: bronchopulmonary foregut
malformations, aberrant luminal recanalization theory, abortive twinning
theory, split notochord theory, intrauterine vascular accident theory.
Most investigators feel that incomplete recanalization of the esophagus
during development is the most likely of the proposed theories.(2)
Three criteria for duplication cysts of the esophagus
are (a) attachment to the esophagus, (b) the presence of 2 layers of muscularis
propria, and (c) the presence of epithelium representing some level of
the gastrointestinal tract. Duplication cysts of the esophagus are usually
lined by ciliated epithelium that is most often columnar but may be flat,
cuboidal, squamous, or pseudostratified. Enteric cysts arise from
the posterior portion of the foregut and contain incomplete muscular layer
on histologic examination. Those cysts which also contain neural tissue
are known as neuroenteric cysts. The latter have connections to the
spinal canal and are invariably associated with vertebral anomalies of
which the most commonly occurring are segmentation abnormalities.(3) In
patients with cystic duplications, the presenting symptoms in early life
may be seen when an expanding mediastinal mass compresses structures in
the mediastinum: (a) vascular compression with cyanosis and engorgement
of head and neck vessels, (b) difficulty in swallowing, regurgitation,
and vomiting, (c) dyspnea, obstructive emphysema, and recurrent respiratory
infections; (d) mediastinitis due to rupture of the duplication. Tubular
duplication may cause recurrent dysphagia and occasional chest pain. When
a complication such as rupture occurs, patients present with signs and
symptoms of acute mediastinitis. Esophageal duplications are more frequently
found in children, but 25% to 30% occur in adults. Men had a 2:1 predominance
over women. The location was 60% in the lower third, 17% in the middle
third, and 23% in the upper third. In true tubular duplications of the
esophagus, esophagography usually shows double-barreled esophagus in which
contrast medium can be seen in both lumina. Contrast enhanced CT scan showed
a low attenuation (21-34 HU) lesion encircling the esophageal lumen through
the entire course of the esophagus. A high-attenuation rim of uniform thickness,
presumed to be the muscular layer of the esophagus. True tubular duplications
must be differentiated from dissecting intramural hematoma of the esophagus
in Boerhaave syndrome with a point of both lumina containing musocal epithelium
as well as muscularis propria.(4) Although radiologic and endoscopic examinations
may suggest the diagnosis, a definitive preoperative diagnosis is difficult.
The definitive treatment of esophageal duplication is surgical excision
and the definitive diagnosis is mainly by the pathologic examination of
the lesion after surgical removal. A 69-year-old alcoholic man was diagnosed
as tubular type of esophageal duplication because of double-barreled
esophagus on esophagogram, low- and high- attenuation rims at the esophagus
on enhanced CT scan, presumed to be the muscular layers of the esophagus.
Histologically, both lumina of the esophagus lined by stratified
squamous epithelium and contained muscularis propria.
References
1. Berk JE, Haubrich WS, Kalser MH, Roth JLA, Schaffner F. Bockus Gastroenterology
4th ed. Saunders, Philadelphia, 1985; 680-681
2. Macpherson RI. Gastrointestinal tract duplications: clinical, pathologic,
etiologic and radiologic considerations. Radiographics 1993; 13: 1063-1080
3. Silverman FN, Kuhn JP. Caffey's Pediatric X-ray Diagnosis: An integrated
imaging approach 9th ed. Mosby, St. Louis 1993; 1008
4. Lee KS, Kim IY, Kim PN. Dissecting intramural hematoma of the esophagus
in Boerhaave syndrome: CT findings. AJR 1991; 157: 197-198