Abstract:A very rare case of noncommunicating multiple intra and retroperitoneal enteric duplication cysts (EDCs) is reported and discussed. Two large noncommunicating EDCs, one within the mesentery of proximal jejunum causing complete luminal obstruction and other isolated cyst in retroperitoneal area displacing duodenum and extrahepatic biliary system, were resected successfully in a 2-day-old neonate along with correction of malrotation.
“…Enteric duplication cysts have been identified and reported as early as 12 weeks into fetal development [10] . Several theories have been proposed on the etiology of enteric duplication cysts: 1) aberrant luminal recanalization theory, which postulates that duplication cysts form as an aberrancy of the developmental canalization process and result in a parallel tract to the normal lumen, 2) abortive twinning theory that represents incomplete twinning during fetal development 3), split notochord theory, 4) persistent embryologic diverticula theory, and 5) sequestration of part of the fetal gut [4] , [11] .…”
HighlightsIntestinal duplications cysts are rare congenital anomalies that can occur throughout the gastrointestinal tract.Isolated intestinal duplication cysts can present with vague abdominal complaints.Diagnosis can be challenging even with imaging.Our clinical workup was suggestive of a mucinous cystic neoplasm (MCN) of the pancreas.This represents the first reported case of an enteric duplication cyst that mimicked an MCN of the pancreas.
“…Enteric duplication cysts have been identified and reported as early as 12 weeks into fetal development [10] . Several theories have been proposed on the etiology of enteric duplication cysts: 1) aberrant luminal recanalization theory, which postulates that duplication cysts form as an aberrancy of the developmental canalization process and result in a parallel tract to the normal lumen, 2) abortive twinning theory that represents incomplete twinning during fetal development 3), split notochord theory, 4) persistent embryologic diverticula theory, and 5) sequestration of part of the fetal gut [4] , [11] .…”
HighlightsIntestinal duplications cysts are rare congenital anomalies that can occur throughout the gastrointestinal tract.Isolated intestinal duplication cysts can present with vague abdominal complaints.Diagnosis can be challenging even with imaging.Our clinical workup was suggestive of a mucinous cystic neoplasm (MCN) of the pancreas.This represents the first reported case of an enteric duplication cyst that mimicked an MCN of the pancreas.
“…Enteric duplication cysts are rare congenital lesions that can originate from anywhere in the GI tract from the oropharynx to the anus, including the retrorectal and presacral space. [1][2][3][4] The key diagnostic features for enteric duplication cysts remain the same regardless of the location and include proximity to the GI tract, a 2-layered coat of well-developed smooth muscle, and a GI-type epithelium. 15,16 Rectal duplication cysts belong to the family of developmental cysts and usually present as unilocular cystic masses located in the prerectal space.…”
Section: Discussionmentioning
confidence: 99%
“…Theories regarding the origin of GI duplications include diverticulization, canalization defects, intrauterine vascular accident, split notochord theory, and the traction hypothesis between the intestinal endoderm and the overlying structures. [1][2][3][4] Enteric duplication cyst is rare, with a prevalence of 1 out of 4,500 individuals, and it may originate from anywhere in GI tract. [5][6][7] Enteric duplication cysts are lined with epithelium similar to that of the adjacent normal GI mucosa and are classified by location as foregut, midgut, or hindgut.…”
“…These include multiple EDCs within one segment of the GT or less frequently in two or more segments (Fig. 4 ) [ 11 , 16 , 18 , 19 ]. Fig.…”
Section: Introductionmentioning
confidence: 99%
“…A vascular insult could have led to the isolation. They are extremely rare [ 19 , 20 ], especially multiple isolated EDCs, which are even rarer [ 19 ].…”
Enteric duplication cysts (EDCs) are rare congenital malformations formed during the embryonic development of the digestive tract. They are usually detected prenatally or in the first years of life. The size, location, type, mucosal pattern and presence of complications produce a varied clinical presentation and different imaging findings. Ultrasonography (US) is the most used imaging method for diagnosis. Magnetic resonance (MR) and computed tomography (CT) are less frequently used, but can be helpful in cases of difficult surgical approach. Conservative surgery is the treatment of choice. Pathology confirms the intestinal origin of the cyst, showing a layer of smooth muscle in the wall and an epithelial lining inside, resembling some part of the gastrointestinal tract (GT). We review the different forms of presentation of the EDCs, showing both the typical and atypical imaging findings with the different imaging techniques. We correlate the imaging findings with the surgical results and the final pathological features.Teaching Points• EDCs are rare congenital anomalies from the digestive tract with uncertain pathogenesis.• More frequently, diagnosis is antenatal, with most EDCs occurring in the distal ileum.• Ultrasonography is the method of choice for diagnosis of EDCs.• Complicated EDCs can show atypical imaging findings.• Surgery is necessary to avoid complications.
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