Enteric duplication cysts in children: varied presentations, varied imaging findings

Abstract

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.

Rare Case of an Isolated Enteric Duplication Cyst in a Teenager

“…They are seen in 1:4,500 births, and found in 0.2% of all children, with a slight male predominance. [3] Intestinal duplications are most commonly detected in infancy and early childhood when symptoms such as abdominal pain, obstruction, or vomiting develop. [4] Some duplication cysts may remain asymptomatic untill adulthood.…”

“…The postoperative recovery and follow up period was uneventful. [3] Excision should be considered in all cases wherever possible. The surgical approach varies depending on the location and type of cysts, with resection and anastomosis being needed in some cases as per the operating surgeons preference.…”

Congenital and hereditary cystic diseases of the abdomen

“…Autosomal recessive polycystic kidney disease -Enlarged kidneys with thickened hyperechoic parenchyma caused by microcysts -Larger cysts (>1 cm) may accompany in some cases -Suggestive findings of hepatic abnormalities including congenital hepatic fibrosis, Caroli disease, and bile duct ectasia Multicystic dysplastic kidney disease -Unilateral cysts in disorganized pattern completely replacing the renal parenchyma, which may be observed on antenatal US (d) Nephronophthisis and medullary cystic kidney disease -Early stage: Hyperechoic renal parenchyma with the loss of corticomedullary differentiation -Advanced stage: Cysts, of varying size, in medullary and corticomedullary locations. The kidneys appear small due to parenchymal fibrosis Gastrointestinal tract * [24][25][26] Duplication cysts -Cyst within the close proximity of the bowel segment -The double wall sign (inner hyperechoic mucosa and outer hypoechoic muscularis propria) -"Y configuration" that is indicative of a shared wall with the cyst and the neighboring bowel wall -Internal septation or luminal debris may be observed due to the infection Lymphatic system * [27,28] Lymphatic malformations -Well-circumscribed cystic lesion with internal septations -The fluid content of the lesion may contain fat -Small lesions may change location on follow-up imaging…”

“…Histopathologically, GI tract duplication cysts consist of an epithelial lining containing the mucosa of the GI tract and a surrounding smooth muscle. The cyst is also closely attached to the enteric wall [24].…”

“…27). This imaging appearance is due to the inner hyperechoic mucosa and the surrounding hypoechoic smooth muscle layer (muscularis propria) [24]. The GI tract duplication cysts share a common wall with the adjacent gut segment.…”