Enteric Duplication Cyst Containing Squamous and Respiratory Epithelium: An Interesting Case of a Typically Pediatric Entity Presenting in an Adult Patient

Abstract

Enteric duplication cysts are rare congenital malformations that can occur at any point along the digestive tract, most commonly the small bowel. They are characterized by the presence of an outer layer of smooth muscle and an inner lining of mucosa that may resemble any portion of the digestive tract. Less commonly, cases have been reported that also contain mucosal components of nonintestinal origin. This entity is typically diagnosed in young children, but occasionally presents in adolescence and young adulthood. We present a rare case of a 21-year-old male who presented with nonspecific symptoms of abdominal discomfort and weight loss and was later found to have a 9 cm nonenhancing mass in the distal ileum on CT imaging. Laparoscopic dissection of the mass revealed a cystic lesion lined mainly by pseudostratified ciliated columnar respiratory-type epithelium, with patchy areas of squamous epithelium as well as villous columnar epithelium resembling small bowel. The unique histology and advanced patient age make this case a unique presentation of what is already a rare pathological entity.

Foregut duplication cyst

“…CT is best for evaluating the shape, extent, and relationship with surrounding structures, [ 4 ] but the detection and diagnosis of enteric cysts itself can still be difficult because the mass can appear as a nonspecific nonenhancing or slightly enhancing one-layered cystic lesion. [ 5 ] In our case, however, the FDC presented like herniated bowel instead of a cystic mass. This CT finding might be confused with the delayed presentation of a congenital diaphragmatic hernia such as a Bochdalek hernia.…”

Histopathological diagnostic dilemma in retrorectal developmental cysts: report of a case and review of the literature

“…In reports related to this topic, the cyst epithelium has similar features to those written in classical textbooks in most of the cases. However, there are reports describing ileal duplication cysts lined by gastrointestinal, respiratory, or patchy-form squamous epithelia in the literature (12). While the cyst surface was eroded in most of the areas in our case, it was lined by columnar and respiratory epithelium in focal areas and a single and organized muscle layer was observed under the surface epithelium in most of the areas.…”

“…Imaging techniques such as ultrasonography, computerized tomography and magnetic resonance imaging are performed for the differential diagnosis of retrorectal cystic lesions. However, the differential diagnosis is quite difficult by radiological methods (12) and the definitive diagnosis is made by histopathological examination, in association with the clinical and radiological findings (12,13).…”

54‐cm enteric duplication cyst in a 13‐year‐old female

“…Etiology of the EDC is unknown4, 5, 8; however, the accepted theories that may explain its pathophysiology are “persistence of the fetal gut diverticula” and “defect in the recanalization of the primitive gut” 4. Evolution of duplication cyst may be due to a failure of the alimentary canal transition from a solid stage into a tube during the 6th and 8th weeks of fetal life 4…”

“…The patient may be symptomatic or asymptomatic 7. The symptoms vary according to the defect site; it may include dysphagia,8 anorexia, vomiting, diarrhea, abdominal pain and distention 9. Some cases—as have been reported—may present with EDC complications, for example, infection, perforation,2 malignant transformation,2, 10 acute intestinal obstruction,5 rectal bleeding,6 intussusception,9 and volvulus 11…”