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Duplication cyst of the pylorus: a case report
© Trainavicius et al.; licensee BioMed Central Ltd. 2013
Received: 17 January 2013
Accepted: 23 May 2013
Published: 5 July 2013
Pyloric duplication is an extremely rare gastrointestinal tract malformation in neonates. This is the first case report of pyloric duplication in our country (Lithuania).
We report the case of a 2-day-old Lithuanian girl who suffered from pyloric duplication mimicking an alternative common bile duct cyst or other intra-abdominal organs cysts. A laparotomy was performed and the cystic formation of the pyloric area was successfully resected. The postoperative course was uneventful.
There are only a few reports describing abdominal masses caused by pyloric duplication mimicking common bile duct cyst or other intra-abdominal organs cysts. Therefore thorough clinical and instrumental examination is needed to determine the most accurate diagnosis that allows one to choose the right treatment.
The term “intestinal duplication” was first used by Fitz, but was not widely used until popularized by Ladd in 1937 . Alimentary tract duplications are very rare and occur in approximately 1 out of 4500 births . True pylorus duplications are extremely rare and present in approximately 2.2% of all alimentary tract duplications . The female-to-male ratio is 2:1 [4, 5]. Although digestive tract duplications are rare, small intestines are the most common location of malformations. Pyloric cystic duplication is the rarest alimentary tract duplication and the literature describes only a few cases. Duplication of the pylorus clinically manifests signs of gastric obstruction, making it difficult to distinguish from hypertrophic pyloric stenosis. The precise diagnosis of pyloric duplication is rarely made before surgery, because this alimentary tract malformation is extremely rare and difficult to perceive. The main method of treatment is radical resection.
A duplication cyst of the pylorus is an extremely rare congenital anomaly of the gastrointestinal tract [6–9]. Duplications may occur in any part of the digestive tract , but they are most commonly found in the ileum, and rarely found in the pyloric area . Pyloric duplications make up 2.2% of all alimentary tract duplications . Of other gastrointestinal abnormalities and vertebral defects, 50% are accompanied by alimentary tract duplications . This anomaly was first described by G.S. Ramsay in 1957 [11, 12], and since then only a few cases of pyloric duplications have been described [6, 12]. This is the first time this anomaly has been reported in Lithuania. The etiology of duplications is unclear. Bremer’s theory suggests that errors in recanalization occur during the fifth to sixth week of gestation, when epithelial proliferation obliterates the lumen of the intestines. Vacuoles form and coalesce to reestablish the lumen of the intestine. Duplications cause failure of these vacuoles to coalesce . Of digestive tract duplications, 85% do not communicate with the gastrointestinal lumen . Cysts near the pylorus, as in our case, are known to present in the neonatal period . However, there are two gastrointestinal tract duplication cases reported in a 41-year-old and a 64-year-old patient . Persistent vomiting is the main symptom of cysts near the pylorus, which occurs due to pyloric obturation [2, 12, 15–18]. Non-bilious vomiting simulating hypertrophic pyloric stenosis should be distinguished from a cyst near the pylorus [6–9]. The usual presentation of duplication of the pylorus is with abdominal mass, vomiting, weight loss and bleeding from the alimentary tract . In older children, it may present with abdominal pain, gastrointestinal bleeding, fever, hemorrhage and perforation leading to peritonitis [19, 20]. The diagnosis of pyloric duplication is difficult because of its rarity. Abdominal ultrasonography, contrast CT, nuclear magnetic resonance, X-ray and fibroesophagogastroduodenoscopy of the gastrointestinal tract facilitate the differential diagnosis, but the disease is most commonly diagnosed during surgery. The main method of treatment is the radical resection of the duplication without injury to the lumen of the bowel.
Duplication cyst of the pylorus is an extremely rare congenital anomaly of the gastrointestinal tract.
Thorough clinical and instrumental examination helps to determine the most accurate diagnosis and allows one to choose the right treatment.
A written informed consent was obtained from the parents of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
The authors thank the Children’s Surgery Centre hospital service members who helped to conduct our patient’s work-up. We thank the National Centre of Pathology that contributed the histopathological examination of this case report. We also thank the Radiologist and Specialists in echoscopy who helped to determine the right diagnosis of this case.
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