Enteric duplication cyst resembling umbilical cord cyst

Abstract

We report a case of a neonatal male with a prenatal diagnosis of umbilical cord cyst that was finally diagnosed as an ileal duplication cyst after postnatal surgical exploration of the umbilical mass. To our knowledge, a similar case has never been reported previously. Although this form of presentation of enteric duplication cyst is exceptional, it should be taken into consideration when evaluating suspected umbilical cord cysts.

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

“…While rare, malignancies arising within duplication cysts typically portend a poor prognosis, as most are diagnosed at advanced stages with widespread metastases. Reported cases have included squamous cell carcinoma, adenocarcinoma, and carcinoid tumors [7, 10]. In this case, the ileal location, nonspecific presenting abdominal symptomatology and absence of concomitant malignancy are consistent with the majority of cases of enteric cysts.…”

“…Enteric duplication cysts are malformations of the digestive system, occurring most commonly in the small intestine, with the ileum representing up to 70% of cases [7]. By definition, cysts must contain at least a single outer muscular layer along with an inner lining of gastrointestinal mucosa, though cysts lined by multiple types of epithelia in addition to gastrointestinal epithelium have been reported [9].…”

“…By definition, cysts must contain at least a single outer muscular layer along with an inner lining of gastrointestinal mucosa, though cysts lined by multiple types of epithelia in addition to gastrointestinal epithelium have been reported [9]. They typically derive both their vascular supply and their smooth muscle wall from the adjacent intestinal segment, though they more rarely have an independent blood supply [1, 7]. Complications include perforation, intussusception, bowel obstruction from adjacent pressure or mass effect, volvulus, and associated malignancy [8].…”

“…The resection of the adjacent normal bowel wall is required due to the sharing of both a muscular wall and a vascular supply, though isolated removal of the cyst alone is possible in the rare cases that have an independent blood supply [18]. When the segment of affected bowel is too large to resect without risking short bowel syndrome, alternative treatment options include selective stripping of the affected intestinal mucosa [7, 19]. In patients with these anomalies an early accurate diagnosis is essential, because subsequent appropriate surgical treatment can lead to a satisfactory outcome.…”

“…Duplication cysts can occur in the context of other congenital abnormalities, such as those of the vertebral bodies and genitourinary tract, though they most often are seen in patients without other developmental malformations [2–4]. The lesions are typically present in the first year of life (70% of cases) or early childhood, though they more rarely have been reported in older patients [5–7]. Initial symptomatology is dictated by the size and location of the lesion and most commonly includes obstruction, with or without vomiting, abdominal distension, dysphagia, palpable mass, or, more infrequently, urinary symptoms [4, 5, 8].…”