Clinical Imaging 40 (2016) 244–246
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Complete gastric duplication in an adult with associated anomalies☆ Zeeshaan S. Bhatti a,⁎, Michelle A. Anderson b, Ashish P. Wasnik a a b
Department of Radiology, University of Michigan Health System, Ann Arbor, MI 48109 Department of Internal Medicine, Division of Gastroenterology, University of Michigan Health System, Ann Arbor, MI 48109
a r t i c l e
i n f o
Article history: Received 6 September 2015 Received in revised form 5 November 2015 Accepted 10 November 2015 Available online xxxx Keywords: Complete gastric duplication Communicating gastric duplication Double stomach Congenital upper gastrointestinal anomalies
a b s t r a c t Gastric duplication is a rare congenital developmental abnormality usually encountered in children. Nearly all reported cases of this entity describe noncommunicating-type duplication cysts. We present an extremely rare case of complete gastric duplication, involving two separate gastric lumens with proximal and distal communication, incidentally discovered in an elderly male in association with various congenital anomalies not previously reported in literature, as diagnosed with computed tomographic scan and upper gastrointestinal endoscopy. © 2015 Elsevier Inc. All rights reserved.
1. Case A 61-year-old man presented with intermittent postprandial left upper quadrant fullness and nausea after meals for several months. Physical examination was unremarkable. A computed tomographic (CT) scan was performed with oral and intravenous contrast for evaluation. CT examination demonstrated two separate gastric lumens (S1 and S2) diverging at the fundus and converging at the pylorus consistent with complete gastric duplication. Additional findings that were noted included annular pancreas with absent distal pancreatic body and tail, multiple left upper quadrant splenules, and incomplete rotation of the duodenum (Figs. 1 and 2). There was also reversed orientation of superior mesenteric artery (SMA) and superior mesenteric vein (SMV) (Fig. 1B). Note was made of a 2.0 cm×1.5 cm cystic lesion in the anterior pancreatic head (Fig. 1B). An endoscopic ultrasound was eventually performed to evaluate the pancreatic cyst, and endoscopy confirmed the complete gastric duplication seen as two separate gastric lumens that communicated proximally at the fundus and distally at the pylorus (Fig. 3). Endoscopic guided pancreatic head cyst aspiration revealed thick mucin with no malignant cells. The patient was managed conservatively. 2. Discussion Duplication of the gastrointestinal tract is a rare congenital developmental malformation, with a reported incidence of approximately 1 in ☆ Disclosure: The authors disclose no conflict of interest. ⁎ Corresponding author. Department of Radiology, University of Michigan Health System, 1500 E. Medical Center Drive, B1 D502, Ann Arbor, MI 48109, USA. Tel.: +1-734232-9048; fax: +1-734-764-2412. E-mail address:
[email protected] (Z.S. Bhatti). http://dx.doi.org/10.1016/j.clinimag.2015.11.016 0899-7071/© 2015 Elsevier Inc. All rights reserved.
4500 live births [1]. Several embryologic mechanisms accounting for the formation of gastrointestinal duplications have been proposed, including aberrant luminal recanalization, partial or abortive twinning, split notochord theory, persistent embryologic diverticula, and intrauterine vascular accidents [2]. However, no single hypothesis can adequately account for all known variants of gastrointestinal duplication [2]. Duplications can occur anywhere along the gastrointestinal tract; the ileum is the most common site of duplication, followed by the esophagus [2]. Duplication involving the stomach, as presented in this case, is very uncommon, accounting for only 4–8% of gastrointestinal duplications [1,3]. Gastrointestinal duplication can be cystic or tubular in configuration and may or may not communicate with the alimentary canal. Cystic duplications typically do not communicate with the gastrointestinal lumen, whereas tubular duplications usually do [4]. Noncommunicating-type cystic gastrointestinal duplication, referred to as duplication cysts, is the most common form, accounting for more than 80% of cases [2]. In contrast, communicating-type tubular duplications are very uncommon and typically have a single communication. Duplications that have two separate sites of communication (proximal and distal), thereby forming a parallel alimentary canal with a second separate lumen, can be defined as complete duplication. Complete duplication anywhere along the gastrointestinal tract is extremely rare. A review of 281 cases of gastrointestinal tract duplications revealed only a single case of complete duplication, which involved the pylorus [2]. Complete duplication involving the stomach, as presented in our case, is exceedingly rare. A review of literature yielded only three reported cases of complete gastric duplication [3,5,6] and a single case of combined complete esophagogastric duplication [7]. In contrast to the tendency of duplication elsewhere along the gastrointestinal tract to occur along the mesenteric border, gastric
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Fig. 2. Coronal reformatted contrast-enhanced CT image demonstrates two gastric lumens (S1 and S2), incomplete rotation of the duodenum (D), and pancreas (P) adjacent to the second portion of the duodenum.
gastric duplication cysts in adults is typically incidental on imaging performed for vague abdominal symptoms, although clinical manifestation may occasionally arise from complications including hematemesis, infection, compression, or carcinoma arising in the cyst [11]. In contrast to gastric duplication cysts, complete gastric duplications are typically discovered incidentally in asymptomatic adults, as in our case. Given the very limited number of reported cases of complete gastric duplication, it is not well known whether this entity is associated with any specific symptoms or complications. Theoretical clinical implications could possibly include acid reflux, peptic ulcer disease, delayed satiety with weight gain, volvulus, and increased risk for gastric cancer. Approximately 50% of cases of gastric duplication are associated
Fig. 1. (A) Axial contrast-enhanced CT abdomen demonstrates complete gastric duplication with the two lumens (S1 and S2) communicating at the pylorus and multiple left upper quadrant accessory spleens (AS). (B) Axial contrast-enhanced CT abdomen demonstrates reversed orientation of SMA and SMV, annular pancreas (arrowhead), and a small pancreatic head cyst (arrow).
duplication is usually found along the greater curvature, corresponding with a dorsal origin relative to the primitive gut during development [8]. Only 5.5% are encountered along the lesser curvature of the stomach [8]. The mucosal lining of the duplicated stomach usually shares a common blood supply with the stomach and resembles normal stomach histologically. However, ectopic lining from other segments of the alimentary and respiratory tracts has been described [9]. The clinical presentation of gastric duplication depends on the size, location, and presence or absence of communication with the gastric lumen. Gastric duplication cysts, which are noncommunicating by the definition mentioned above, are reportedly nearly twice as common in adult females [2]. Given its rarity, the gender predilection of complete gastric duplication is not well described. Patients with gastric duplication cysts typically present in infancy, with nearly 67% of cases discovered within the first year of life [3]. Clinical presentation usually involves nonspecific symptoms including nausea, vomiting, abdominal pain, mobile palpable epigastric mass, gastrointestinal bleeding (due to ectopic gastric mucosa secreting acid causing ulceration), weight loss, and failure to thrive [4]. Rarely, the duplication may undergo torsion, result in obstruction, or lead to perforation [10]. Diagnosis of
Fig. 3. Antegrade endoscopic view centered at the fundus shows two gastric lumens (S1 and S2) at the proximal site of communication.
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with other congenital anomalies, including duplication or atresia elsewhere along the alimentary canal, vertebral or spinal cord malformations, pulmonary abnormalities, and pancreatic abnormalities (e.g., pancreatic duplication, aberrant pancreatic duct, accessory pancreatic lobe) [4,6,12,13]. The variety of coexistent pancreatic abnormalities reported in the setting of gastric duplication reflects the common foregut origin of the stomach and pancreas; an inciting developmental anomaly in the foregut is thus linked to the concomitant anomalies in both. For example, it has been postulated that neurenteric band formation during embryogenesis results in traction diverticula that give rise to gastrointestinal cysts and additionally interfere with pancreatic development [14]. To our knowledge, our case is the only reported case in English literature of complete gastric duplication associated with annular pancreas, absence of distal body and tail of pancreas, and incomplete rotation of the duodenum. The exact correlation and association of these abnormalities remains uncertain given the limited literature on this rare anomaly. Historically, upper gastrointestinal (UGI) series has played a major role for the diagnosis of gastric duplication. UGI series may demonstrate extrinsic compression or displacement of the stomach and adjacent bowel loops by the duplication and/or communication between the duplication and adjacent viscera [15]. In the case of complete gastric duplication, UGI series can adequately demonstrate a double-lumen stomach, with two separate courses of enteric contrast running in parallel extending from the gastroesophageal junction to the pylorus, where both lumens converge [6]. Ultrasound of gastric duplication can demonstrate peripheral gut signature characterized by an inner thin echogenic mucosal layer surrounded by a hypoechoic muscular layer (“double wall” or “muscular rim” sign). Gastric duplication cysts contain a wide anechoic central portion due to their cystic nature. On the other hand, communicatingtype duplications can demonstrate a discernible communication tract on ultrasound, with the duplication containing echogenic air and/or debris similar to the enteric contents seen elsewhere within the gastrointestinal tract [16]. On CT, gastric duplication cysts appear as fluid-filled cystic masses with thick, mildly enhancing wall in close proximity to the stomach. Focal areas of high attenuation within these cysts are representative of hemorrhage or proteinaceous material, whereas focal enhancement may be indicative of infection or malignant degeneration [2]. In the case of complete gastric duplication, CT can help delineate detailed anatomical configuration, including the extent of duplication, its relationship with neighboring organs, and associated anomalies. 99Tc scintigraphy has high specificity in identifying gastric mucosa and has been successfully utilized in the diagnosis of gastric duplication [17]. Because noncommunicating-type gastric duplications often present with symptoms or portend the risk of complications including malignancy, they are typically treated surgically via complete resection of the duplication or, alternatively, excision of the shared wall between
the duplication and the normal stomach [13]. Complete duplication of the stomach does not require surgical intervention when both lumens are patent. 3. Conclusion While gastric duplication (most commonly seen as noncommunicating cysts) in itself is a rare subset of gastrointestinal tract duplications, it is very rare to encounter communicating-type gastric duplication and exceedingly rarer to encounter complete gastric duplication. This case of complete gastric duplication adds to the very few reported cases available in English literature, illustrating that complete gastric duplication can be an incidental finding in adults. In addition, this case is the first to demonstrate other associated congenital findings not previously described in literature. References [1] Puligandla PS, Nguyen LT, St-Vil D, Flageole H, Bensoussan AL, Nguyen VH, et al. Gastrointestinal duplications. J Pediatr Surg 2003;38:740–4. [2] Macpherson RI. Gastrointestinal tract duplications: clinical, pathologic, etiologic, and radiologic considerations. Radiographics 1993;13:1063–80. [3] Blinder G, Hiller N, Adler SN. A double stomach in an adult. Am J Gastroenterol 1999; 94:110–1102. [4] Iyer CP, Mahour GH. Duplications of the alimentary tract in infants and children. J Pediatr Surg 1995;30:1267–70. [5] Di Pisa M, Curcio G, Marrone G, Milazzo M, Spada M, Traina M. Gastric duplication associated with pancreas divisum diagnosed by a multidisciplinary approach before surgery. World J Gastroenterol 2010;16:1031–3. [6] Agha FP, Gabriele OF, Abdulla FH. Complete gastric duplication. AJR Am J Roentgenol 1981;137:406–7. [7] Mazziotti MV, Ternberg JL. Continuous communicating esophageal and gastric duplication. J Pediatr Surg 1997;32:775–8. [8] Takahara T, Torigoe T, Haga H, Yoshida H, Takeshima S, Sano S, et al. Gastric duplication cyst: evaluation by endoscopic ultrasonography and magnetic resonance imaging. J Gastroenterol 1996;31:420–4. [9] Theodosopoulos T, Marinis A, Karapanos K, Vassilikostas G, Dafnios N, Samanides L, et al. Foregut duplication cysts of the stomach with respiratory epithelium. World J Gastroenterol 2007;13:1279–81. [10] Koumanidou C, Montemarano H, Vakaki M, Pitsoulakis G, Savvidou D, Kakavakis K. Perforation of multiple gastric duplication cysts: diagnosis by sonography. Eur Radiol 1999;9:1675–7. [11] Kuraoka K, Nakayama H, Kagawa T, Ichikawa T, Yasui W. Adenocarcinoma arising from a gastric duplication cyst with invasion to the stomach: a case report with literature review. J Clin Pathol 2004;57:428–31. [12] Ueda U, Taketazu M, Itoh S, Azuma H, Oshima H. A case of gastric duplication cyst with aberrant pancreas. Pediatr Radiol 1991;21:379–80. [13] Ildstad ST, Tollerud DJ, Weiss RG, Ryan DP, McGowan MA, Martin LW. Duplications of the alimentary tract: clinical characteristics, preferred treatment and associated malformations. Ann Surg 1988;208:184–9. [14] McLetchie NGB, Purvies JK, Saunders RC. The genesis of gastric and certain intestinal diverticula and enterogenous cysts. Surg Gynecol Obstet 1954;99:135–41. [15] Faerber EN, Balsara R, Vinocur CD, de Chadarevian JP, et al. Gastric duplication with hemoptysis: CT findings. AJR Am J Roentgenol 1993;161:1245–6. [16] Yang DM, Kim HC, Choi SI, Lim SJ. Sonographic diagnosis of gastric duplication cyst communicating with the gastric lumen. J Clin Ultrasound 2011;39:550–2. [17] Dittrich JR, Spottswood SE, Jolles PR. Gastric duplication cyst: scintigraphy and correlative imaging. Clin Nucl Med 1997;22:93–6.