Massive unilocular intraabdominal cysts in children are rare. Alimentary tract duplications can present diagnostic and therapeutic difficulties. Although they can occur anywhere from the mouth to the anus, they are commonly seen in relation to the ileum. We herein present an unusual case of duplication cyst itself occupying virtually all of the available intraabdominal volume. It appeared to be an enteric duplication cyst of ileal origin.
A 3-month-old girl was admitted to our hospital for investigation of progressive abdominal distension and biliary vomiting. Plain radiography of the abdomen showed normal air-fluid level in the stomach and paucity of gases in rest of the abdomen. Magnetic resonance imaging showed a huge, homogenous cyst extending from the xiphisternum down to the pelvis. The cyst was excised completely. Macroscopic examination and histologic findings confirmed the diagnosis of a huge enteric duplication cyst arising from the ileum.
Enteric duplication cyst should be considered in a patient with an abdominal cystic mass. Radiologist must take into account patient age, clinical parameters, and imaging findings to identify the likely etiology of a cystic mass.
abdominal cystic masses; enteric duplication cyst; magnetic resonance imaging; plain radiography
A 21 day old male child presented with non bilious vomiting and abdominal mass.
This case is reported because pyloroduodenal duplication cysts are an extremely rare congenital anomaly, whose clinical presentation often mimics those of hypertrophic pyloric stenosis. Ultrasound examination showed cystic mass at pyloric region and barium study was suggestive of extrinsic mass compressing the pyloric region. A laparotomy, a tense cystic mass was present at the pyloroduodenal junction (PDC) which was resected and end to end anastomosis was done. Patients followed an uneventful recovery and doing well.
The clinical and radiological analysis can reveal configurational changes consistent with a large extrinsic mass rather than muscular hypertrophy and can lead to accurate preoperative diagnosis.
•Gastrointestinal stromal tumors (GIST) are solid tumors.•Atypical presentations in form a cystic lesion have been rarely reported.•A duodenal GIST masquerading as a cystic lesion has never been reported.•First report a case of a large duodenal cyst that finally, turned out to be a GIST.•Successfully managed by local resection avoiding a pancreaticoduodenectomy.
Gastrointestinal stromal tumors (GIST) are solid tumors. A duodenal GIST masquerading as a cystic lesion has never been reported. We report a large duodenal cyst that finally turned out to be a GIST and was managed without a pancreaticoduodenectomy (PD).
Presentation of case
A 55 year old lady presented with painful lump in epigastrium. A CT scan revealed a large exophytic cystic lesion from the duodenum with a small solid component. An endoscopy showed a polypoid lesion in the second part of the duodenum adjacent to what looked like a diverticulum. A sleeve duodenal resection, duodeno-duodenostomy and pyloric exclusion was done. The histopathology was duodenal GIST.
This case posed diagnostic difficulty as it was thought to be either a duplication cyst or a diverticulum of duodenum. The odd point was the small solid component in it. We considered the possibility of a malignancy arising in these settings, which is has been occasionally reported. To our surprise, it turned out to be a GIST. An extensive literature search yielded only four reports that have reported cystic GISTS, all arising from the stomach or pancreas where they have been mistaken for pseudocysts or even a mucinous cystadenocarcinoma of the pancreas. This is the first report of a cystic GIST arising from the duodenum.
GISTS can present as a predominantly cystic lesion and needs to be considered in the differential diagnosis of cystic lesions of the duodenum. Local resection is an attractive option in select cases and avoids a PD.
Gastrointestinal tumor; Cystic degeneration; Duodenal cyst; Duodenal diverticulum; Pyloric exclusion
Duodenal duplication is a rare congenital malformation and has been reported as a rare cause of recurrent acute pancreatitis. Hemorrhagic ascites has been reported in only one case of duodenal duplication.
An 11-year-old Chinese girl presented with abdominal pain, hematemesis and dark stools. On admission, an abdominal examination revealed a moderately distended abdomen with diffuse tenderness. Biochemical investigations showed increased serum levels of amylase, lipase, and urine amylase. An abdominal computed tomography scan and magnetic resonance imaging scan revealed an enlarged and heterogeneous pancreas with poorly delineated borders. There was a cystic lesion measuring 25mm × 48mm × 28mm, located between the descending portion of her duodenum and the head of her pancreas. There were massive effusion signals in her abdominal cavity. An exploratory laparotomy was performed. A tubular cyst measuring 32mm × 52mm × 30mm was found in the second part of the duodenum, next to the head of her pancreas. The anterior wall of the duplication cyst was resected and anastomosis of the remaining cyst to the duodenum was performed for drainage. Histopathological examination of the excised cyst wall showed duodenal mucosa, submucosa and muscle coats, indicative of a duodenal duplication.
It is important to be aware of duodenal duplication when evaluating a patient with recurrent acute pancreatitis accompanied by massive hemorrhagic ascites.
Acute pancreatitis; Child; Duodenal duplication; Hemorrhagic ascites
Duplication cysts (DCs) of alimentary tract are rare congenital malformations. They are firmly attached to the wall of the gastrointestinal tract and they are supplied by surrounding mesenteric blood vessels. More than 80% of cases occur before the age of two years and only a minority of cases present in adulthood. “Completely isolated duplication” of the alimentary tract is an extremely rare variety of gastrointestinal duplications. They have gastrointestinal epithelial and wall characteristics without an anatomic association with the alimentary tract. Their main characteristic is that they have their own blood supply. A 20-year-old male was admitted to our department with symptoms persisting for a period of one week prior to admission, which included abdominal pain, fever, and a palpable abdominal mass. CT revealed an unexplained intraperitoneal abscess. This case represents a rare clinical example of infected isolated duplication cyst managed with percutaneous drainage and surgical excision of the cyst 3 weeks later. To the best of our knowledge, this is the first reported case to use this approach.
Background. Duplication of the alimentary tract is a rare congenital anomaly. Gastric duplication cysts (GDCs) represent 4% of all alimentary tract duplications, and approximately 67% manifest within the first year of life. Duplication cysts in adults are generally encountered as incidental findings at endoscopy or laparotomy. Herein, we report two rare cases of symptomatic GDC presenting in adults. Case 1. A 27-year-old male presented with a five-month history of back pain. Exam revealed mild epigastric tenderness with a vague palpable mass in left upper abdomen. CT scan showed 8 × 7.4 × 6 cm homogenous, nonseptated cystic mass posterosuperior to pancreatic tail. On laparotomy, a cystic mass measuring 11 × 8 cm was found, which was densely adherent to posterior wall of stomach suggestive of GDC. Case 2. A 28-year-old woman presented with epigastric pain associated with vomiting for 2 months. Exam revealed mild epigastric tenderness. CT scan showed four cystic lesions in the medial wall of distal stomach measuring approximately one cm each suggestive of duplication cysts. Exploratory laparotomy with antrectomy and truncal vagotomy with Billroth II reconstruction were performed. Pathology in both patients was diagnostic of GDC. Conclusion. GDC is a rare anomaly, and its presentation in adults is even rarer.
Intra-abdominal esophageal duplications are rare entities in adults. They are mostly asymptomatic, but since they can lead to complications surgical excision is advised for all duplication cysts. We present a case of a 20-year-old male with colic-like abdominal pain, mimicking symptoms of cholecystolithiasis. However after cholecystectomy the symptoms were still present. A computed tomography-scan of the abdomen and an endoscopic ultrasound revealed a cyst of the esophagus of 3.0 cm × 2.3 cm in size. Diagnostic laparoscopy was planned, during which we observed a para-esophageal cyst at the gastro-esophageal junction. Laparoscopic excision of this cyst was performed. Pathophysiological examination revealed an esophageal duplication cyst. We report a rare case of a symptomatic intra-abdominal esophageal duplication cyst in an adult. One must consider this diagnosis when more common diagnoses to account for the patient’s symptoms are excluded. Removal of duplication cysts can be done laparoscopically.
Esophageal duplication cyst; Intra-abdominal; Symptomatic; Laparoscopy
Enteric duplications are rare congenital anomalies of the digestive tract that can occur anywhere along its length, with the majority being found in the small intestine. The duodenum is the least common site. Almost all symptomatic duodenal duplications present early in life with abdominal pain and pancreatitis. To the best of our knowledge this is the first described case of a perforated tubular duodenal duplication in an elderly adult.
PRESENTATION OF CASE
We present a case of a perforated tubular duodenal duplication in an elderly woman. She presented with diffuse abdominal pain, fever, and tachycardia. Emergent exploratory laparotomy revealed a perforated duodenal duplication. Excision of the duodenal duplication and primary closure of the defect was performed successfully. The patient recovered well.
Enteric duplications are poorly understood anomalies of embryonic development. They can be cystic or tubular dorsal enteric remnants lying in communication with the alimentary tract that are distinct from diverticula. A tubular duodenal duplication is exceedingly rare, and this case is made even more notable in that such an anomaly presented with sepsis and occurred in a 79 year old woman. We are unsure why the duplication ruptured. To the best of our knowledge this case represents the first report of a ruptured tubular duodenal duplication in an elderly adult.
This is a very rare occurrence and has never been described in an elderly patient before. Excision and primary closure led to a good outcome.
Duplication; Duodenum; Perforation; Duodenal; Elderly; Abdomen
Gastric duplication cysts are rare variety of gastrointestinal duplications. Sometimes they may present with complications like hemorrhage, infection, perforation, volvulus, intussusception and rarely neoplastic changes in the gastric duplication cyst. We present one and half year old male child who developed sudden abdominal distension with pain and fever for two days. Ultrasound revealed a cystic mass in the hypochondrium and epigastric regions. On exploration an infected and perforated gastric duplication cyst was found. Surgical excision of most part of cyst wall with mucosal stripping of the rest was performed. Histopathology confirmed the diagnosis of gastric duplication cyst. Early surgical intervention can result in good outcome.
Gastric duplication cyst; Acute abdomen; Peritonitis
Duplication cysts are rare gastrointestinal congenital abnormalities and can occur anywhere within the gastrointestinal tract. Duplication cysts are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply, and they do not communicate with the intestine. These cysts are usually diagnosed during early childhood, and very rarely detected in adults, mostly incidentally, due to a lack of symptoms. A 28-year-old male was admitted to our hospital with a chief complaint of lower abdominal pain and distention and a palpable mass for 1 month. Based upon computed tomography and sonographic findings, a small bowel duplication cyst was tentatively diagnosed. The cyst had no connection to the gastrointestinal tract. Herein we report the case of a noncommunicating isolated ileal duplication cyst in an adult. Resection of the cyst was performed safely without requiring bowel resection.
Duplications of the alimentary tract are very rare. A one-month-old female presented with symptoms of anorexia, vomiting and continuous watery diarrhea. The plain abdominal radiograph showed thickened intestinal wall and signs of small bowel obstruction. The fevers, vomiting, and continuous wartery diarrhea persisted despite antibiotics, and worsened. The patient failed to respond to medical managements, 27 hours after admission, the patient died due to multiple organ failures. The autopsy was performed, small bowel obstruction due to an ileocecal duplication cyst (3 × 3 cm) was recognized. The ileocecal duplication cyst was attached to the ileum which was changed edematous and necrotic. This potential diagnosis should be borne in mind for a patient who complains of abdominal symptoms with an unknown cause, and duplication cyst should be recognized as a fatal cause in infant.
sudden unexpected death; small bowel obstruction; ileocecal duplication cyst
Duplication of the alimentary tract is a rare congenital malformation that occurs most often in the abdominal region, whereas esophageal duplication cyst develops typically in the thoracic region but occasionally in the neck and abdominal regions. Esophageal duplication cyst is usually diagnosed in early childhood because of symptoms related to bleeding, infection, and displacement of tissue surrounding the lesion. We recently encountered a rare adult case of esophageal duplication cyst in the abdominal esophagus. A 50-year-old man underwent gastroscopy, endoscopic ultrasonography, computed tomography, and magnetic resonance imaging to investigate epigastric pain and dysphagia that started 3 months earlier. Imaging findings suggested esophageal duplication cyst, and the patient underwent laparoscopic resection followed by intraoperative esophagoscopy to reconstruct the esophagus safely and effectively. Histopathological examination of the resected specimen revealed two layers of smooth muscle in the cystic wall, confirming the diagnosis of esophageal duplication cyst.
Intestinal duplications are rare developmental anomalies that can occur anywhere along the gastrointestinal tract. Rectal duplication cysts account for approximately 4% of all duplication cysts. They usually present in childhood with symptoms of mass effect, local infection or more rarely with rectal bleeding from ectopic gastric mucosa.
PRESENTATION OF CASE
A 26 year old male presented with a history of bright red blood per rectum. On examination a mucosal defect with an associated cavity adjacent to the rectum was identified. This was confirmed with rigid proctoscopy and CT scan imaging. A complete transanal excision was performed.
Rectal duplication cysts are more common in pediatric patients. They more frequently present with symptoms of mass effect or local infection than with rectal bleeding. In adult patients they are a rare cause of rectal bleeding. Definitive treatment is with surgical excision. A transanal, transcoccygeal, posterior sagittal or a combined abdominoperineal approach may be used depending on anatomic characteristics of the duplication cyst.
We present a rare case of a rectal duplication cyst presenting in adulthood with rectal bleeding, managed with transanal excision.
Rectal duplication cyst; Adult; Rectal bleeding
Gastric duplication cysts comprise 2-7% of gastrointestinal duplications, rare congenital malformations that can be present at almost any part of the alimentary tract. They mostly present with gastrointestinal obstruction symptoms, ulceration, and painless hemorrhage. Symptoms include nausea, vomiting, and fullness sensation. Gastric duplications are mostly cystic in shape. Herein, we present a 58-year-old man with a gastric duplication cyst, 70×30×35 mm in size, with the initial presentation of abdominal pain, icterus, and elevated liver enzymes. The patient provided informed consent for this report.
Jaundice; Elevated liver enzymes; Abdominal pain
Duplication of the alimentary tract may occur in any site, from the mouth to the anus, and in recent years such anomalies have received wide notice. Pyloric duplication cyst is an extremely rare congenital anomaly of the alimentary tract, whose clinical presentation often mimics those of hypertrophic pyloric stenosis. Gastrointestinal duplications are observed in 1 of every 4500 autopsies, predominantly in white males. It represents 2.2 % of all gastric duplications, with only 18 have been reported up to 2011 (Table 1). In most cases preoperative diagnosis is not made. We report a case of a pyloric duplication cyst in a 3-year-old girl with progressive increased vomiting. The patient had an ultrasonography, upper gastrointestinal series, and computer tomography of the abdomen. The diagnosis was confirmed by surgery and histopathology examination. The patient was asymptomatic at 12-month follow-up. The clinical and radiological analysis can reveal configurational changes consistent with a large extrinsic mass rather than muscular hypertrophy and can lead to accurate preoperative diagnosis.
Pyloric duplication; Hypertrophic pyloric stenosis; Gastric outlet obstruction; Enucleation; Pyloroantrectomy
A 4-year-old girl presented with non-bilious vomiting and loss of appetite and weight. At laparotomy, a non-communicating pyloroduodenal duplication cyst was present. Subtotal excision of the cyst and cauterization of the mucosal lining of the common wall was performed. The post-operative recovery was uneventful.
Gastric duplication; gastric outlet obstruction; pyloroduodenal duplication
Duplication cysts are congenital cystic malformation of the alimentary tract consisting of a duplication of the segment to which it is adjacent. It can occur anywhere from mouth to anus. Oesophageal duplication cysts comprise 4% of the same. Of these, total intra-abdominal oesophageal duplication cysts are extremely rare. On review of literature, only 3 case reports of total intra-abdominal oesophageal duplication managed laparoscopically are found. All these cases were adults. We report the first paediatric case of intra-abdominal oesophageal duplication cyst excised laparoscopically.
Duplication cyst; oesophageal; laparoscopy
About 10% to 15% of all duplication cysts in the alimentary tract are esophageal. Esophageal duplication cysts are intimately attached to the alimentary tract, are lined by mucous membrane and have smooth muscle. This paper describes a 2-year-old child who presented with symptoms of progressive respiratory distress. A diagnosis of esophageal duplication cyst was made. At surgery a low cervical incision was made and the sternal manubrium split, thereby providing adequate exposure. The cyst was then removed. The most useful investigations were chest roentgenography and barium esophagography. Computerized tomography showed a small, round foreign body in the middle of the cyst that was subsequently found to be a bingo chip. Communication between the cyst and the esophagus was not obvious at the time of surgery and had not been demonstrated by barium esophagography. When complete excision of the cyst is not possible because of inflammatory reaction all the mucosa must be removed to prevent recurrence. Careful postoperative respiratory support and broad-spectrum antibiotic therapy are recommended.
Enteric duplication cysts are rare and uncommon congenital malformations formed during the embryonic period of the development of human digestive system and are mainly encountered during infancy or early childhood, but seldom in adults. The clinical presentation is extremely variable depending upon its size, location and type. We present six cases of enteric duplication cysts with diverse clinico-pathological features.
Materials and Methods
This study was carried out in the Department of Pathology and Department of Paediatric Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India for a period of 2 years (January 2013 - December 2014). We retrospectively analyzed six patients of enteric duplication cysts based on data obtained, which consisted of patient’s age, sex, clinical presentation, radiological features, operative findings and histopathology report. The data collected was analyzed by descriptive statistics.
Six children between age range of 3 days to 10 years had enteric duplication cysts. Two had ileal and one each were of pyloroduodenal, colonic and rectal duplication cyst. In one patient a presumptive diagnosis of enteric duplication cyst was made. Radiology played an important contributory role in diagnosis of these cysts in all the patients but histopathology proved to be gold standard for its confirmation. All these patients were managed by surgical excision. The postoperative and follow up period in all the cases was uneventful.
It is important to be aware and make a definitive diagnosis of this rare congenital anomaly as they can present in various clinical forms and can cause significant morbidity and even mortality if left untreated by causing life threatening complications.
Congenital anomaly; Histopathology; Surgical excision
Intestinal duplication is an uncommon congenital condition in young adults. A 25-year-old man complained of chronic, intermittent abdominal pain for 3 years following previous appendectomy for the treatment of suspected appendicitis. Abdominal discomfort and pain, suggestive of intestinal obstruction, recurred after operation. A tubular mass was palpable in the right lower quadrant. Computed tomography enterography scan identified suspicious intestinal intussusception, while Tc-99m pertechnetate scintigraphy revealed a cluster of strip-like abnormal radioactivity in the right lower quadrant. On exploratory laparotomy, a tubular-shaped ileal duplication cyst was found arising from the mesenteric margin of the native ileal segment located 15 cm proximal to the ileocecal valve. Ileectomy was performed along with the removal of the duplication disease, and the end-to-end anastomosis was done to restore the gastrointestinal tract continuity. Pathological examination showed ileal duplication with ectopic gastric mucosa. The patient experienced an eventless postoperative recovery and remained asymptomatic within 2 years of postoperative follow-up.
Ileal duplication cyst; Adulthood; Computed tomography enterography; Tc-99m pertechnetate scintigraphy
Gastric duplication cyst is a very rare gastrointestinal tract malformation that accounts for 2%-4% of alimentary tract duplications. Most cases are diagnosed within the first year of life, following presentation of abdominal pain, vomiting, and weight loss and clinical discovery of an abdominal palpable mass. This case report describes a very uncommon symptomatic gastric duplication cyst diagnosed for the first time in adulthood. Only a few other case reports of similar condition exist, and all were identified by endosonography. The current case involves a 52-year-old male who presented with a one-month history of progressive iron deficiency anemia without overt gastrointestinal bleeding. The patient underwent esophagogastroduodenoscopy, which revealed a 2.0 cm pinkish subepithelial lesion, suspected to be a gastrointestinal stromal tumor (GIST) and source of gastrointestinal bleeding. The endosonography showed inhomogeneous hypoechoic lesions with focal anechoic areas arising from a second and third layer of the gastric wall. Differential diagnoses of GIST, neuroendocrine tumor, or pancreatic heterotopia were made. The lesion was removed using an endoscopic submucosal resection technique. Histopathology revealed an erosive gastric mass composed of a complex structure of dilated gastric glands surrounded by fibro-muscular tissue, fibroblasts, and smooth muscle bundles, which led to the diagnosis of gastric duplication.
Gastric duplication cyst; Gastrointestinal bleeding; Endoscopy; Endoscopic ultrasonography; Endoscopic mucosal resection; Differential diagnosis; Adult
Enteric duplication (ED) is a rare congenital anomaly that can occur anywhere along the alimentary tract from the mouth, down to the anus and the nearby organs. This uncommon anomaly may be asymptomatic or presents with vague symptoms mimicking other common pathologies. We aim to present our experience, management challenges and patterns of ED with a review of the literature.
Settings and Design:
The study was carried out at a Nigerian Tertiary Hospital (2005–2008 inclusive).
Materials and Methods:
We retrospectively analyzed seven patients with ED managed in our hospital for sex, age, clinical presentations, duplication size and site, presence of ectopic tissue, complications, associated anomalies, radiological workups, and prognosis. Data was analyzed using SPSS 11.0 for window.
Seven children between the age range of 44 hours–10 years had ED, one sublingual and six intraabdominal duplications. Midgut volvulus with long segment bowel gangrene complicated two cases. The diagnosis was incidental in all. Three cases were diagnosed following conventional radiological contrast examination and the rest at surgery. Ultrasound was not helpful in making diagnosis in all the six intraabdominal duplications. Though surgery was recommended for all, one of the patients declined. Only one patient had unsuccessful surgery.
ED requires high index of clinical suspicion and careful management. Many cases of nonspecific abdominal pains should be properly evaluated before patients suffer avoidable complications.
Bowel gangrene; children; enteric duplication; surgery; volvulus
Alimentary tract duplications are rare congenital anomalies. The presentation depends on their anatomical location, size and other characteristics. The most common variety is small bowel cystic duplication. We report a case of an eight years old girl who presented with recurrent abdominal pain and melena. Radioisotope technetium scan showed increased uptake of tracer in right lower abdomen and a diagnosis of Meckel’s diverticulum made. At surgery a cystic, communicating, ileal duplication found which was resected along with adjacent gut. It is thus reiterated that while investigating children with recurrent abdominal pain and melena, gut duplications must be included in the differential diagnosis.
Alimentary tract duplications; Pain abdomen; Melena
Duplication of alimentary tract is one of the rare congenital anomalies. A case of duplication cyst of the cecum, presented in the 3rd month of life, as intestinal obstruction. Excision of the cyst along with cecum and appendix was done. The child had an uneventful postoperative recovery following ileoascending anastomosis.
Caecal duplication cyst; Intestinal obstruction
Concurrence of duodenal atresia and gastric duplication cyst is extremely rare entity. We report a 6-day-old female neonate who presented with neonatal intestinal obstruction. X-ray abdomen showed double bubble sign. At laparotomy, a huge cystic structure attached to greater curvature of the stomach along with duodenal atresia of second part of duodenum was found. The cystic structure was excised and duodeno-duodenostomy performed. Histopathology report confirmed it gastric duplication cyst.
Gastric duplication; Duodenal atresia; Neonate