Thoracic Duplication Cysts of the Alimentary Tract
Borislav A. Alexiev, M.D.
Allen P. Burke, M.D.
Terminology
Esophageal and enteric duplication cysts are foregut-derived developmental anomalies most commonly encountered in the mediastinum and rarely in the abdomen.1,2,3,4,5,6,7,8 The duplication of the hollow structures of the digestive tract manifests three essential points: (1) attachment to at least one point of the alimentary tract, (2) epithelium resembling some part of the alimentary tract, and (3) welldeveloped coat of smooth muscle (double-layered smooth muscle wall).7,9,10
Intramural esophageal cysts result from defects in the tubulation process and are not associated with vertebral anomalies.7 In contrast, enteric cysts are usually separate from the esophagus and lie in the posterior mediastinum. They are commonly associated with vertebral anomalies and are part of split notochord syndrome.1,9
Incidence and Clinical
Among alimentary tract duplication cysts, 20% occur in the mediastinum.9 The majority of duplications are found in children, the frequency being almost triple to that found in adults.9 The commonest mode of presentation in the neonatal period is respiratory distress. In adults, they can be discovered as an incidental finding on chest radiograph. About two-thirds of alimentary tract cysts are right sided.7
Gross Pathology
Intramural esophageal and enteric cysts are unilocular. The lesions are thin walled and translucent, with a tan, smooth, and glistening outer surface. The inner lining of the cyst is smooth, without papillary projections. The cysts contain pink serous or yellow-gray mucinous fluid.
Microscopic Pathology
Intramural esophageal cysts contain at least two types of epithelium, including ciliated or nonciliated columnar, squamous, or a mixture of these types, heterotopic lung tissue, thyroid stroma, ganglia, and two smooth muscle layers.6,7,10,11,12 Heterotopic gastric mucosa and well-differentiated lymphoid aggregates resembling Peyer patches are rarely observed.