ESOPHAGEAL DUPLICATION CYST
Clinical history
A 49-year-old man presented with recurrent episodes of vague upper abdominal pain. History revealed chronic obstructive pulmonary disease. Physical examination and laboratory tests were irrelevant. Endoscopy of the esophagus, stomach and duodenum was normal. Ultrasonography (not shown) and CT scan of the abdomen were performed, followed by endosonography of the upper GI tract.
Radiological diagnosis
CT scan of the abdomen after IV and oral administration, at the level of the cardia
shows a rounded lesion of low attenuation (arrow) localized posteriorly to the left liver lobe adjacent to the anterolateral wall of the esophagus (curved arrow).
CT scan at the level of the pancreas demonstrated a 2.8 cm rounded solid lesion in the pancreatic tail with similar attenuation values as pancreas and spleen (arrow). The lesion is poorly demarcated from pancreatic tissue.
On endosonograpphy through the stomach there is a well delineated solid lesion in the pancreatic tail (arrow). No abdominal lymph nodes are seen. At the level of the distal esophagus there is a well delineated cystic lesion into the esophageal wall, just outside the muscle layer (not shown).
Explorative laparotomy with resection of the lesion was performed. The following
figure shows the macroscopic aspect of the resected pancreatic tail.
At the same time a cyst of the outer layer of the distal esophagus was enucleated.
Histology of the resected specimen revealed pancreatic tissue and well encapsulated normal splenic tissue (spleniculus), without signs of inflammation or malignancy. Histology of the lesion near the distal esophagus disclosed a lining of ciliated respiratory epithelium, surrounded by a wall of collagen, muscle and fatty tissue, suggestive for a duplication cyst.
Discussion
Accessory spleens are found in 10% of the population. They usually occur in the gastrosplenic ligament, but may be found in the pancreas, GI tract and even scrotum.
Approximately 15% of the ectopic spleens are found in the pancreatic tail. Spleniculi are histologically identical to the normal spleen. They arise early in embryonic life from failure of complete coalescence of normal primordial splenic buds. They are seldom larger than 1.5 cm in diameter and are usually asymptomatic.
Esophageal cysts may occur in the wall of the esophagus or in the paraesophageal region. They may be congenital (rare) or acquired.
Embryologically they result from developmental errors occurring in the fifth to eight week of life. Esophageal duplication cysts are most frequently intramural lesions. They can be lined with respiratory, enteric, gastric or squamous epithelium. Histological differentiation between esophageal, bronchogenic and neurogenic cysts is very difficult. Clinical presentation is variable, most cysts are asymptomatic. Those attached to the esophagus and protruding into the posterior mediastinum may cause respiratory symptoms. Cysts from the lower third of the esophagus may produce dysphagia, nausea, vomiting and weight loss.