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CASE REPORT Table of Contents   
Year : 2014  |  Volume : 11  |  Issue : 3  |  Page : 276-278
Noncommunicating multiple intra-abdominal enteric duplication cysts

1 Department of Surgery, Division of Paediatric Surgery, Sultan Qaboos University Hospital, Muscat, Oman
2 Department of Child Health, Division of Neonatology, Sultan Qaboos University Hospital, Muscat, Oman
3 Department of Radiology, Sultan Qaboos University Hospital, Muscat, Oman

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Date of Web Publication22-Jul-2014


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.

Keywords: Enteric duplication cyst, gut development, malrotation of gut, retroperitoneal cyst

How to cite this article:
Mandhan P, Ehsan TM, Al-Sibai S, Khan AM, Sankhla D. Noncommunicating multiple intra-abdominal enteric duplication cysts. Afr J Paediatr Surg 2014;11:276-8

How to cite this URL:
Mandhan P, Ehsan TM, Al-Sibai S, Khan AM, Sankhla D. Noncommunicating multiple intra-abdominal enteric duplication cysts. Afr J Paediatr Surg [serial online] 2014 [cited 2021 Oct 28];11:276-8. Available from:

   Introduction Top

Enteric duplication cysts (EDCs) are rare (1 in 4,500), congenital anomalies that may originate anywhere in the gastrointestinal tract (GIT). [1] EDCs are hollow, epithelial-lined, and cystic or tubular structures that are attached with the wall of the gut and supplied by mesenteric vessels. EDCs are more common in small bowel, either run parallel to the small bowel (tubular) or may lie within the mesentery (spherical) and may or may not be communicating with the adjacent bowel segment. [2] EDCs in retroperitoneum are very rare and till date only seven cases have been reported in English literature. [3],[4],[5],[6],[7],[8] These cysts are isolated, do not communicate with GIT, and have their own blood supply. [9] Concomitant occurrence of intra and retroperitoneal EDCs is extremely rare and as per knowledge has not been reported yet. We present first case of noncommunicating intra and retroperitoneal EDCs associated with malrotation of gut in a 2-day-old neonate.

   Case Report Top

A preterm twin, 35-week gestation age, was delivered in our hospital. Antenatal scan showed bubble stomach and there were three intra-abdominal cystic lesions near to the stomach. At birth, baby's APGAR was 6 at 1 min and 8 at 5 min. He was transferred to neonatal intensive care unit (NICU) for monitoring and observation. His abdominal examination was unremarkable and he passed small amount of meconium immediately after delivery. He had moderate volume of light yellow color aspirate from his nasogastric tube. His plain abdominal X-ray showed dilated stomach, duodenum, and proximal small bowel with no gas in the rest of the abdomen [Figure 1]. His clinical and radiological findings were consistent of proximal small bowel obstruction (small bowel atresia). He underwent laparotomy, which showed a 5 × 7 cm fluid filled cyst in the mesentery of proximal jejunum, compressing small bowel lumen and causing complete obstruction [Figure 2]. There was another 6 × 3 cm isolated cyst located in the right upper quadrant, which was also fluid-filled and extending in the retroperitoneum, displacing the duodenum forward. The cyst was also causing anterior displacement of the extrahepatic biliary system but not occluding the lumens of ducts. The retroperitoneal cyst had a small vascular pedicle coming from mesenteric vessels. The caecum and appendix were malpositioned, lying in the midline. The proximal jejunal mesenteric cyst was excised along with the attached piece of bowel followed by an end-to-end anastomosis. The isolated retroperitoneal cyst was also excised completely [Figure 3] and Ladd's procedure was performed to correct malrotation. To confirm the communication between jejunal mesenteric cyst with the lumen of resected jejunal segment attached with cyst, a size 8 Fr feeding tube was passed from one end to other and no communication was noted [Figure 3]. Patient's postoperative course was uneventful. An ultrasound abdomen was done a week postoperative to delineate hepatobiliary anatomy and also to document any residual/additional retroperitoneal cysts, which did not reveal any abnormality. The histological examination of both cysts revealed cyst wall lined by well formed small bowel type mucosa, submucosa, circular, and longitudinal layers of muscularis mucosa confirming small bowel EDCs.
Figure 1: Plain X-ray of abdomen showing gas distended stomach and duodenum; and no evidence of gas filled bowel loops distal to it

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Figure 2: Enteric duplication cyst located at the mesenteric border of proximal jejunum

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Figure 3: Completely resected retroperitoneal enteric duplication cyst (top) and enteric jejunal duplication cyst including resected segment of jejunum and feeding tube is passed through the jejunal segment showing no communication of gut with the cyst (bottom)

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   Discussion Top

EDCs are rare congenital anomalies, which can occur anywhere in the GIT from mouth to anus, although there is greater predilection towards the ileal region. [10] The hallmark of diagnosis is the close proximity of the cyst to some part of the alimentary tract with sharing its blood supply. In isolated EDCs, there is no definite communication or connection with the nearby GIT. However, the presence of the typical histopathological features like distinct muscle wall layers and lining epithelium would qualify for the diagnosis of noncommunicating duplication cysts. [11] In our case, the findings correlate with the above explanations of EDCs, as we encountered two EDCs, one situated within the mesentery of proximal jejunum with no luminal communication, and other cyst was located in retroperitoneal area with its separate vascular pedicle and not connected with the nearby gut segment.

Embryogenesis of alimentary tract duplications cysts is unclear. Several hypotheses for formation of EDCs have been postulated including diverticulization, canalization defects, intrauterine vascular accident theory, split notochord theory, and the traction hypothesis between intestinal endoderm and the overlying structures. [12],[13],[14] These do not explain the formation of extraperitoneal EDCs. We propose that a part of fetal gut may get sequestered at a very early stage of development in the extraperitoneal space such as retroperitoneum or chest to develop into retroperitoneal or mediastinal EDC and take its vascular supply from gastrointestinal vascular tree. We also suggest that in intraperitoneal EDCs, occurrence of malrotation of gut may be a contributory factor by causing lymphatic obstruction of a part of embryonic gut, thus resulting in formation of noncommunicating cyst. This hypothesis may well explain the occurrence of two noncommunicating EDCs at two different anatomical locations in our patient, one in the retroperitoneum with separate blood supply and other within the mesentery of small bowel along with associated malrotation of gut.

The clinical presentation of EDCs depends on anatomical location, size, and inner lining of the cyst wall. In previously reported seven cases of retroperitoneal EDCs, 60% of these cysts were picked up and managed in neonatal period [3],[6],[10] , whereas the remaining 40% cases presented late with delayed complication of obstruction and jaundice in adulthood. [4],[8],[9] In all these cases, the cysts were located in upper left quadrant and contained gastric mucosa. Compared to previously reported cases, in our patient the location of retroperitoneal EDC was on right side close to duodenum and did notcontain gastric mucosa. Unlike other previously reported cases of retroperitoneal EDCs, in our case, there was a second noncommunicating circular EDC in the mesentery of proximal jejunum and also associated malrotation of gut.

The treatment of all intra and extraperitoneal EDCs is complete resection either by open transperitoneal or retroperitoneal approaches. Recently laparoscopic techniques have been used successfully in the excision of intraperitoneal EDCs in infants. [15] However, in our case we used open transperitoneal approach to completely excise two EDCs at a time.

Awareness of varied types and possible anatomical locations of EDCs is essential and would be helpful to choose optimal early surgical intervention.

   References Top

1.Kim YJ,Kim YK, Jeong YJ, Moon WS, Gwak HJ. Ileal duplication cyst: Y-configuration on in vivo sonography. J Pediatr Surg 2009;44:1462-4.  Back to cited text no. 1
2.Cheng G,Soboleski D,Daneman A,Poenaru D,Hurlbut D. Sonographic pitfalls in the diagnosis of enteric duplication cysts. AJR Am J Roentgenol 2005;184:521-5.  Back to cited text no. 2
3.Duncan BW, Adzick NS, EraklisA. Retroperitoneal alimentary tract duplications detected in utero. J Pediatr Surg 1992;27:1231-3.  Back to cited text no. 3
4.LoYS, Wang JS,Yu CC,Chou CP,Chen CJ,Lin SL, et al. Retroperitoneal enteric duplication cyst. J Chin Med Assoc 2004;67:479-82.  Back to cited text no. 4
5.Lund DP. Alimentary tract duplications.In:O'Neill JA Jr, Rowe MI, Grosfeld JL, editors. Pediatric Surgery.St. Louis: Mosby. p. 1389.  Back to cited text no. 5
6.May DA, Spottswood SE,Ridick-Young M,Nwomeh BC. Case report: Prenatally detected dumdbell-shaped retroperitoneal duplication cyst. Pediatr Radiol 2000;30:671-3.  Back to cited text no. 6
7.Schalamon J, Schleef J,Höllwarth ME. Experience with gastro-intestinal duplications in childhood. Langenbecks Arch Surg 2000;385:402-5.  Back to cited text no. 7
8.Takiff H, Brems JJ, Elliott ML. Calcified retroperitoneal enteric duplication cyst. Am J Gastroenterol 1993;88:470-1.  Back to cited text no. 8
9.Upadhyay N, Gomez D, Button MF,Verbeke CS,Menon KV. Retroperitoneal enteric duplication cyst presenting as a pancreatic cystic lesion. A case report. JOP 2006;7:492-5.  Back to cited text no. 9
10.Pachl M, Patel K,Bowen C,Parikh D. Retroperitoneal gastric duplication cyst: A case report and literature review. Pediatr Surg Int 2012;28:103-5.  Back to cited text no. 10
11.Okamoto T, Takamizawa S,Yokoi A,Satoh S,Nishijima E. Completely isolated alimentary tract duplication in a neonate. Pediatr Surg Int 2008;24:1145-7.  Back to cited text no. 11
12.Favara BE, Franciosi RA,Akers DR. Enteric duplications. Thirty-seven cases: A vascular theory of pathogenesis. Am J Dis Child 1971;122:501-6.  Back to cited text no. 12
13.Menon P, Rao KL, Vaiphei K. Isolated enteric duplication cysts. J Pediatr Surg 2004;39:e5-7.  Back to cited text no. 13
14.Steiner Z,Mogilner J. A rare case of completely isolated duplication cyst of the alimentary tract. J Pediatr Surg 1999;34:1284-6.  Back to cited text no. 14
15.FordWD, Guelfand M,López PJ,Furness ME. Laparoscopic excision of a gastric duplication cyst detected on antenatal ultrasound scan. J Pediatr Surg 2004;39:e8-e10.  Back to cited text no. 15

Correspondence Address:
Dr. Parkash Mandhan
Department of Surgery, Division of Paediatric Surgery, Sultan Qaboos University Hospital, Al Khod 123, Muscat
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0189-6725.137344

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  [Figure 1], [Figure 2], [Figure 3]


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